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Resorptive arthropathy and the opera-glass hand syndrome
S eminars
in and Rheumatism
Arthritis
1972-1973
VOL. II, NO. 3
Resorptive
Arthropathy
the Opera-Glass Robert Stanley
L. Swezey. J. Alexander,
Hand David
and
Syndrome
M. Bjarnason,
and D. 6. Forrester
A
N INCREASING AWARENESS of the diversity of entities associated with a mutilating arthritis and the opera-glass hand (or the main en lorgnette syndrome) has led to a need for more precise categorization of resorptive arthropathies and their differentiation from other destructive skeletal disorders of the hands and feet. Since the first description of a case of resorptive arthropathy in 1888, there have been only scattered reports over the subsequent years. Although a variety of diseases have been associated with bone resorption, only a few demonstrate a resorptive process primary in the joint, characteristically in the hands and feet, and unassociated with clinically demonstrable neural or vascular defects. These cases in which the resorptive process not only caused a marked destruction of articular surfaces sufficient to cause a pleated appearance in the redundant loose overlying skin, but a remarkable tapering and “whittled” appearance of the shafts of the bone as well are considered to have primary resorptive arthropathy and include only certain of the collagen diseases and multicentric reticulohistiocytosis. To clarify this new classification, a review of the historical descriptions of resorptive arthritis in rheumatoid arthritis and in rheumatoid variants in association with the main en lorgnette syndrome, will be briefly summarized. A description of the clinical, pathological, and radiographic findings of the primary causes of resorptive arthritis and the main en lorgnette syndrome will be outlined, and contrasted with the findings in the other destructive arthritides and resorptive disorders of bone.
Supported
in part by a grant from the Arthritis
Foundation.
Robert L. Swezey, M.D.: Associate Professor of Medicine and Physical Medicine and Rehabilitation, University of Southern California School of Medicine. David M. Bjamason, M.D.: Fellow in Rheumatology, University of Southern Calijornia School of Medicine. Stanley J. Alexander, M.D.: Assistant Clinical Professor of Medicine, University of Southern California School of Medicine. D. B. Forrester, M.D.: Assistant Professor of Radiology, University of Southern Calijbrnia School of Medicine. 01973 by Grune & Stratton, Inc.
Seminars
in Arthritis
and Rheumatism,
Vol.
2. No. 3,
1972-l
973
191
192
SWEZEY
ET AL.
DEFINITION
Resorptive* arthropathy is defined as an articular disorder characterized by severe resorption of bone commencing at the articular surface or its margins and spreading proximally and distally. It usually predominantly affects the hands and feet and is typically associated with diffuse regional bony demineralization. It may have, in addition, infolded redundant overlying skin as a consequence of the loss of underlying bone.t (Fig. l-4).
Fig. 1. Case 1. Rheumatoid arthritis-main en lorgnette. skin overlying areas of bony resorption are evident.
Fig. 2.
Gross deformity
Case 7. Rheumatoid arthritis and systemic lupus erythematosis. areas of bony resorption is evident in all fingers.
and pleated
Redundant
redundant
and pleated
skin overlying
*Resorb: to lyse and assimilate (the substance of a differentiated structure previously produced by the body). Webster’s Third New International Dictionary of the English Language-Unabridged Springfield, Massachusetts. G. & C. Merriam Co., 1961. TOpera-glass hand, main or doights en lorgnette.
OPERA-GLASS
193
HAND
Fig. 3. Case 7. Rheumatoid arthritis and systemic lupus erythematosis. Severe resorption of the phalanges of the right fourth toe and associated pleated overlying redundant skin result in a doigt en lorgnette deformity in the foot.
Fig. 4. Case 8. Scleroderma (PSS) and rheumatoid arthritis. Classical main en lorgnette deformity. The skin overlying the digits is redundant but that over the wrist is somewhat thin, and areas of vitiligo can be noted. The skin has a somewhat shiny and unwrinkled appearance over the wrists and proximal dorsal hand area.
HISTORICAL
REVIEW
A review of the literature summarizes the case reports of resorptive arthropathy with sufficient juxta-articular bone resorption in most cases to cause redundancy and pleating of overlying skin (Table 1). The first recorded description of a case of resorptive arthropathy was by Bourdillon in his 1888 doctoral thesis entitled “Psoriasis et Arthropathies.“’ One of his cases showed resorption of several digits with almost complete disappearance of the right fifth toe. In 1903, Adrian reported a second case of psoriasis in a 44-yr-old white male who had severe destruction of the metatarsal and metacarpal phalangeal joints.* In 1906, Brumpt described a 35yr-old retarded Sudanese male with progres-
SWEZEY
ET AL.
: :
a!
3:
h
n2
R
P
7
OPERA-GLASS
HAND
195
196
SWEZEY
ET AL.
sive muscular atrophy and “doigts en lorgnette” or an “opera glass fingers” deformity which progressed with the muscular atrophy. This was the first use of the term “opera glass” to describe the infolded skin overlying resorbed bone. The exact etiology of this case, however, remains obscure.3 The contribution which attracted widespread attention to this curious entity was that of Marie and Leri in 1913 entitled “Une VariCtt de Rheumatisme Chronique: La Main en Lorgnette.” They described in detail the clinical, radiologic, and autopsy findings of a 70-yr-old woman with a 28-yr history of generalized arthritis which resulted in complete ankylosis of carpometacarpal joints, elbows, shoulders, knees, hips, and the vertebral column. Her digits were so resorbed that her hands and feet resembled “paws.” The skin over the digits was folded like an opera glass. Radiographs showed bony “transparency” and bony resorption of all bones of the hands, wrists, and of the distal radius and ulna. The pathology was characterized by fatty replacement of articular structures, some granulation tissue between bony structures, and sparing of tendons, nerves, and blood vessels except for mild endarterial thickening.4 This very complete description leaves only one lingering doubt, and that is whether or not this patient may have had ankylosing spondylitis in light of the mention of a complete ankylosis of the vertebral column in an admitted aged and infirm female. One of Zellner’s cases reported in 1928 was a man with severe psoriasis, typical resorptive arthropathy, generalized joint disease, and ankylosis of the cervical spine. Although the description of this patient is incomplete, as the sacroiliac joints and dorsal spine are not mentioned, it seems likely that this man had psoriasis, resorptive arthropathy, and ankylozing spondylitis. Under the title “La Main en Lorgnette,” Weigeldt,6 in 1929, reported a case similar to that of Marie and Ltri. He was the first to emphasize the tapering of the distal ulna. In 1931, Bauer and Vogl’ described a 64 yr-old male with psoriasis and resorptive arthropathy of the hands and feet. Their patient had typical clinical and radiographic features of ankylosing spondylitis. Blum,8 in 1933, used the title “Osteoarthropathia Mikroatrophicans” and described a 48-yr-old woman who had clinical evidence of subcutaneous nodules, normal serum calcium, phosphorous, uric acid, radiographic evidence of marked bone “atrophy,” and severe destruction of the distal clavicles and elbows, as well as of the metacarpophalangeal and proximal interphalangeal joints. The skin of the hands was loose and wrinkled and looked as though a “too large glove was being worn.” Stursberg,9 in 1935, reported two cases as “Polyarthritis Mutilans.” One had foot involvement which suggests the changes described later in the discussion of Werner’s syndrome. In 1936, Shlionsky and Blake” described an additional patient with psoriasis and resorptive arthritis involving the hands, wrists, and feet. Illustrations of their patient with the main en lorgnette syndrome received wide viewing as a result of having been published by Cornroe.” Nelson’s’* 31-yr-old female patient, reported in 1938, had intercarpal and interphalangeal fusion as well as resorption, cornea1 opacification, a renal calculus, normal serum calcium, phosphorous and phosphatase, and a parathyroid adenoma could not be determined with certainty. NO pathologic description of the joints was given.
OPERA-GLASS
197
HAND
In Schinz’s13 1939 text, a 45-yr-old
white female with “arthritis
mutilans”
in-
volving her hands and feet is illustrated. In 1941, Crain14 reported a 55-yr-old man with a 13-yr history of rheumatoid arthritis associated with marked joint resorption, no ankylosing, and death due to chronic renal disease of undetermined cause. No autopsy was obtained. Von Werthemann,” in 1945, described the pathological articular changes noted at autopsy of a 75yr-old patient consisting of fatty degeneration and minimal arterial involvement similar to that first noted by Marie and L&i. In 1946, Neilsen and Snorrason16 used the title “Arthritis Mutilans (Main et doight lorgnette)” and described the joints of six female rheumatoid arthritics with the syndrome. They noted that none of their patients had psoriasis nor evidence of neurological involvement and commented that in two of their patients there was a suggestion that mechanical factors may have played a secondary role in the joint destruction. in 1950, chose “Chronic Absorptive Arthritis or Solomon and Stecher,” Opera-Glass Hand: Report of Eight Cases” as their title. Two of their patients were psoriatics, one of whom lost the vision in an eye due to iritis. There were three male patients in their series. They introduce the term “cupping” to describe the hollowing out of the base of a bone at the joint surface opposite the tapered erosion of the distal head of the opposing articular surface. They also note, as had others dating back to the report of Marie and L&i, in both psoriatics and nonpsoriatics, the presence of fusion in joints showing severe resorption. They further remarked that joints not involved in the resorption process appeared to be otherwise typical of rheumatoid arthritis. In 1952, Smyth”’ reviewed the literature and reported four additional patients, including the first black (with the exception of the peculiar case of Brumpt). This second patient was noted to have a subcutaneous nodule (this one substantiated as a rheumatoid subcutaneous nodule by biopsy). Of particular additional interest is the autopsy report of a male patient who was described as having Still’s disease at age 23 (age of onset not given) and who died at age 33 of unstated causes. The articular findings at autopsy were reported as follows: “The cartilage and underlying cortical bone is replaced and held together by acellular fibrous tissue and fat. The remaining bony trabeculae were thin and the compact bone showed marked atrophy.” One of Smyth’s cases was also noted to have a unilateral protusio acetabuli (“Otto pelvis”). Serial radiographs over a 14-yr period demonstrated the radiographic manifestations of the pathological sequence of events in one patient. Joint narrowing and minimal erosions were present in a second metacarpophalangeal joint for 9 yr and then over the next 5 yr progressive resorptive cupping of the base of the proximal phalanx was observed. Silver and Steinbrocker” describe five patients, only two of whom had typical resorptive arthropathy of the hands and wrists; the other three demonstrated large cystic lesions located in the knee (distal right femur) in one, in the olecranon process of the ulna in another, and in the proximal head of the humerus in the third. Two of these patients had biopsies; one of the knee and the other of the shoulder, which showed the bone destruction resulted from a direct communication of granulation tissue from the eroded cartilage and bony surface into the bone. There was no evidence of a vascular lesion. Unfortunately, these
198
SWEZEY
ET AL.
biopsied cases were not truly representative of the usual acral form of resorptive arthropathy. “Arthritis mutilans” was the term used by MatherZo in June 1954 to describe a 36-yr-old female with rheumatoid arthritis who, over a two-yr span, developed pain-free hypermobile joints typical of resorptive arthropathy. In addition, she had Sjogren’s syndrome manifested by keratoconjunctivitis sicca, recurrent left parotitis, and a deficiency of tears. Biopsy of a subcutaneous nodule was reported as typical of rheumatoid arthritis. In 1955, Eisenstadt and Eggers” described a 17-yr-old male patient with recurrent heel and foot pain of four years’ duration. Attacks of foot pain were “unbearable” and ultimately a doight en lorgnette deformity of both fifth toes developed. Severe pain in the right knee occurred in the fourth year of his disease. Radiographs showed “cup and pencil” changes of the fifth metatarsophalangeal joint within 1 yr of onset. A year subsequent to this, further erosive changes were noted in the left heel and periosteal elevation of the shafts of the right second and third metatarsals was also observed. No mention is made of the possibility of psoriasis, ankylozing spondylitis, nor of Reiter’s syndrome. A biopsy of both fifth metatarsal joints revealed “yellow-colored cancellous bone partly replaced by a firm, shiny, greyish, soft tissue mass representing an osteolytic process with chronic inflammatory reaction and sclerosis of the blood vessels.” Friedman et a1.,22 in their study of the LE phenomenon in rheumatoid arthritis, described a 62-yr-old white male with psoriasis and several positive LE smears and a sheep cell titer of 1:28 who developed arthritis at age 45, which progressed to severe crippling and resorptive arthropathy by age 47. Their patient manifested ankylozing spondylitis.22 In 1966, Williams, Bland, and Lipson reported on a 74-yr-old woman with long-standing rheumatoid arthritis, a latex agglutination titer positive in a l:lO, 240 dilution, a biopsy-proven rheumatoid nodule, and marked resorptive arthropathy of the phalanges, carpals, proximal radii, distal ulnae, proximal humeri, and distal end of the right clavicle. In addition, and of particular interest, she had marked erosion of the odontoid, posterior displacement of the atlas (attributed to either loss of the anterior arch of the atlas or posterior dislocation of the atlas over the odontoid process with probable loss of its anterior arch), multiple cervical vertebrae subluxations, and a neurologic picture consistent with cervical spinal cord compression.23 Except for the nonspecific findings of an elevated sedimentation rate and a mild anemia, there has been little evidence to suggest a common pathophysiologic sequence of events in these cases of resorptive arthropathy. PRIMARY
Rheumatoid
RESORPTIVE
ARTHROPATHIES
Arthritis
Although resorptive arthropathy was first described in conjunction with psoriasis, it was Marie and Ltri’s description of the “main en lorgnette” syndrome in a patient with rheumatoid arthritis that initiated wide interest in the phenomenon.‘12
OPERA-GLASS
199
HAND
Prior to the widespread
use of the rheumatoid
factor
determinations
and the
ARA diagnostic criteria, there was no specific criterion that could establish a diagnosis of rheumatoid arthritis other than a biopsy-proven rheumatoid nodule.24.25 Only four of 37 patients described as rheumatoid arthritics (excluding the juvenile rheumatoid arthritics, the ankylozing spondylitics, and the cases with psoriasis) had clinical or biopsy-proven rheumatoid nodu1es.8~‘*~20,23 Rheumatoid factor in significant titre was reported in only one of the 37 cases. This patient had a biopsy-proven rheumatoid factor titer of 1:10,000.23 Since this is the only nonpsoriatic case reported since rheumatoid factors have been routinely measured and since the majority of patients with rheumatoid ‘nodules have significant rheumatoid titers, it seems likely that some of the 33 reported rheumatoid patients with resorptive arthropathy in whom no nodules are described represent examples of the usually benign seronegative rheumatoid disease which have developed the main en lorgnette deformity.25~28 Resorptive arthropathy occurs in patients with severe rheumatoid arthritis. The joints not involved in the resorptive process are typical on biopsy, X-ray, and clinical examination of rheumatoid arthritis.4,‘5,‘7,‘” The male/female ratio in rheumatoid arthritis is 0:3330.50: 1 and in resorptive arthritis is 0.55: 1.28(See Table 2.) The duration of disease to the onset of resorptive arthritis ranges from four to 50 yr with an average duration of 15 yr, and the average age of patients at the time of being reported is 46.5 yr. The distinctive clinical features of resorptive arthritis are evident bone resorption with loose and redundant overlying skin in the digits. Radiographitally, one observes dissolution of bone involving primarily the PIP and MCP or MTP joints. The DIP joints may be involved in the resorptive process in rheumatoid arthritis but are more often spared than in the psoriatic cases.‘8s29 The dissolution of bone progresses from the articular surface to create a tapering of the proximal articulating bone (licked candystick or whittled appearance) and a hollowing or cupping of the proximal articular surface of the distal bone. This may be associated with resorption of all or a part of the phalanges of one or more digits (doight en lorgnette).16 Frequently, the distal ulna, elbow, and occasionally the clavicle, shoulder, hip and cervical spine, including spinous processes, are involved.‘4.‘8*23 Compression or destruction of bone by a contiguous rheumatoid nodule has not been a factor in causing resorptive arthritis, although isolated reports of bone compression by this mechanism have been made.30,3’ Bone ankylosis is not an uncommon feature of rheumatoid arthritis occurring most notably in the carpal and tarsal bones, but occasionally in a digit as well. Resorptive changes may be seen in the same extremity manifesting ankylosed interphalangeal joints in both rheumatoids and psoriatics. Thus, new bone production and resorption are not mutually exclusive processes.8.‘8.32 The resorptive process tends to be insidious but, as mentioned, may progress rapidly and be well advanced in a 4-mo period.2’,32 The development of resorptive arthritis is commonly accompanied by a reduction in severity of pain and inflammation in the involved joints.8,20
200
SWEZEY
!lxBm
ET AL.
II
RESORPTIVE AFKt'HROPATHy AND THE OPEFhGLASS HAND SYNDROME
NO.OF CASES DURATION OF ARTHRITIS IN YEARS*
YEARS OF ARTHRITIS Average Range x5.3
4u57
MEAN AGE OF ALL PATIENTS INYEARS
49/57
46
WY++
4S
lS/lS”
45
IBAN
AGE OF FEWLES +
EAN
AGE OF MALES +
WITE
s/50*
RACE JINFh?LlE FJ-IEU?Wl?OlDS INKYLOSING
SPONTZLITIS
34
4 moe.+O yrs.
WQ?GE 21-75
BUCK
2
4 4-
SJOGREN'S SYND3.0~
1
PSORIASIS
16
*
Duration does not necessarily imply duration of arthritis prior to onset of Resorptive Artbropathy.
++
Number of cases in which information is available out of total number of cases reported.
m
Eleven additional cases had either sacroiliitisor ankylosis of vertebrae, but insufficientdata y,yas given to accurately categorize them tither.
+
Male/female ratio = 0.55/l.O
OPERA-GLASS
HAND
201
Because of extensive destruction in the resorptive arthritic process, many patients have become severely crippled; but a common observation is a surprising degree of useful and dextrous function in spite of incredibly deformed hands and other joints, which is probably attributable to the subsidence of articular pain in the destroyed joints.8,2” (‘USCI: (A.B.).(Figs. 5-8) Classical rheumatoid arthritis with high titer rheumatoid Factor and Sjogren’s syndrome. The patient is a 40.yr-old white female who had the onset of monoarticular rheumatoid arthritis involving the right elbow in 1950 at age 20. Over the ensuing 3 mo the disease became generalized, resulting in a nonambulatory status, and she developed a progressive deforming mutilating arthritis of all extremities, particularly the fingers. In 1953, she had surgery on both knees for correction of flexion contractures and postoperatively was able to ambulate with crutches until 1963, at which time she again became confined to a wheelchair. In 1967 she first noticed burning and dryness of her eyes and in May 1969 she was noted to have classical changes of Sjogren’s syndrome on slit lamp examination. In July 1968 she developed cervical cord compression secondary to cervical spine involvement with rheumatoid arthritis whtch was successfully treated conservatively with a cervical collar. Musculoskeletal examination at that time showed generally greater than 50”,, loss in range of motion in all joints, with Rexion contractions of all extremities, ulnar deviation of the fingers, and main en lorgnette deformity of both hands. Neck motion was restricted to 15” of lateral flexion; 35” forward flexion: 15” extension: and 60” of rotation. There was generalized decrease in motor strength. normal deep tendon reflexes, flexor plantar responses, decreased sensation to touch and pain over both arms. and equivocal findings of mild spasticity in the legs. Cervical spine X rays showed destruction of the third and fourth cervical bodies with destruction of the interspace between C2 and C3 and between C4 and C5 and extensive destruction of the posterior spine with slight anterior displacement of the bodies of C2. 3. and 4. Physical examination was otherwise within normal limits. Significant laboratory studies were Hb. 11.7, WBC 9000 (normal differential), ESR 28, albumin 2.5, globulin 3.6. antinuclear antibodies negative, serum complement normal. rheumatoid factor positive in 1:163,840 dilution LE cell preparation negative. Other significant illnesses include a vagotomy and pyloroplasty in April 1967 for a gastrointestinal hemorrhage, a gastroJejeunostomy in March 1969 for a recurrent hemorrhage, and an acute paranoid psychosis in November 1968. Treatment of her rheumatoid arthritis was initially steroids for the first 2 yr and primarily aspirin since that time
Fig. 5. Case 1. Rheumatoid arthritis. Concentric resorption of the phalanges of the second finger has caused foreshortening and overlap of the soft tissues resulting in the classic “opera glass” deformity. Resorption of the second metacarpal head and subluxation of the base of the phalanx add to the deformity. The third and fourth fingers demonstrate an additional cause of destruction. Subluxation of the middle phalanges has caused saucerlike pressure erosions as their abnormally positioned ends erode the proximal phalanges. The wrist demonstrates subluxation as well as resorptive changes of the distal radius and ulna.
SWEZEY ET AL.
Fig. 6. Case 1. Rheumatoid arthritis. Progressively more severe destruction of each digit demonstrates the series of changes that occur in the formation of a pencil and cup deformity. Although the first, second, and third metatarsophalangeal joints appear ankylozed. oblique views verified this to be destruction of cartilage with secondary irregularities of the articular ends of the bones. Resorption of the heads of the fourth and fifth metatarsals has caused widening of the joint spaces. lnvagination of the pencilled metatarsal bones into the base of the fourth and fifth proximal phalanges ultimately produces the pencil in cup deformity.
Fig. 7. Case 1. Rheumatoid arthritis. The mutilating effects of rheumatoid arthritis are not confined to the hands and feet of this patient. Tapering of the radius and ulna and remodeling of the distal humerus wi?h total destruction of the joint are apparent.
203
HAND
OPERA-GLASS
Fig. 8. Case 1. Rheumatoid arthritis. Resorption of the femoral head and protrusion of the neck into the pelvis may reflect the tendency toward generalized resorption of bone. A superimposed avascular necrosis cannot be excluded without a series of films to document the progression of pathological events.
(V.B.)
(‘use 2:
(Figs. 9 and 10) Classic rheumatoid
arthritis
with resorptive
anthropathy
mal in the hands, but remarkable
in the cervical spine. The patient
the onset of rheumatoid
in 1954 at age 43. The disease began in her shoulders,
arthritis
wrists, and hands and progressed to generalized present, she has had persistent she developed
subcutaneous
Sjogren’s syndrome
moderately
nodules
active
around
since 1963. Treatment
both
Of significance
I4 consisting of arthritis, examination
multiple ecchymotic examination sternal border
skeletal examination
radiation
heart
palmar grade
creattnine
I. I, gamma-globulin
0.6,
with
erythema;
albumin
2.6,
component,
severe rheumatoid
motion,
of both knees, swelling
factor I /2560, normal, with
anti-DNP
ulnar
resorptive
left
deviation
interphalangeal
joints,
motion
ol
surgical scars.
alpha*-globulin antibody
the
Musculo-
IO” Rexion contracture
bilateral
platelets
chest X ray normal. early
negative.
On with
HEENT
along
of the eyes showed
differential), 0.5.
140/80.
and decreased
joints with overlying
6900 (normal
arthritis
abdomen
metatarsal
symptoms.
heard
joints and proximal
alpha’-globulin
74 mg Y;, ECG
murmur
ol
fever at age
appearance
blood pressure
systolic
Slit lamp examination
12.3, WBC
2.0, rheumatoid
tion positive, cryoglobulin compatible
was normal.
tests: Hb.
symptoms
5 yr of her disease
cushinoid
of the neck and shoulders;
of the metacarpophalangeal
examination
normal.
a moderately
II/VI
and with no diastolic
of ths hips, synovial thickening
Laboratory
in her disease,
She has never had any neurological
both ankles, and swelling of the metatarsophalangeal Neurological
for the initial
swelling of both wrists with decreased
of the fingers, subluxation
keratitts.
aspirin
presented
revealed decreased motion
of both elbows, synovial normal motion
and chorea.
1969, the patient
chest negative,
without
Early
elbows,
Up to the
5-10 mg day). In 1964 she had bilateral
skin lesions and bilateral
negative,
relapse.
and she has manifested
in her past history is an episode of acute rheumatic
carditis,
in November
elbows
mini-
female who had
over a short period of time.
disease without
was mainly
and then steroids for the past IO yr (Prednisone head resections.
involvement
is a 59-yr-old
tilamentary
normal, 0.8.
urinalysis
beta-globulin
negative,
LE cell prepara-
X-rays
of the hands were
changes.
The
cervical
spine
204
SWEZEY
ET AL.
Fig. 9. Case 2. Rheumatoid arthritis. Generalized cartilage destruction of the metacarpophalangeal joints and erosive changes of the proximal and distal interphelangeal joints have resulted in severe malalignment, and a wavy contour of the fingers mimicking an “opera glass” hand. Resorptive changes of the proximal phalanges of the thumbs and the distal ulna are also present.
revealed marked resorption of the spinous processes of CS. In November 1965 the patient developed a large ulcer on her lower right leg after a cat scratch, which slowly healed after conservative treatment. Case 3: (V.L.) (Fig. 1 I) Seronegative rheumatoid arthritis, Sjogren’s syndrome, and resorptive arthropathy. The patient is a 72-yr-old black female who had the onset of rheumatoid arthritis in 1930 at age 31. The disease began in her thumbs and progressed to generalized joint involvement over the next few years. By the time she was first seen in our clinic in 1966, she had a supracondylar osteotomy of the right knee. She has had Sjogren’s syndrome since 1967. In June 1969 she had an episode of iritis. Over the past 4 yr, she has complained of 223 hr of morning stiffness with intermittent paints and occasional swelling in various joints. She has never had a subcutaneous nodule. She has had no neurologic symptoms. Medication over the years has been mainly aspirin and she has never had gold treatment. Examination in August 1966 was as follows. Examination of the head, chest, heart, and abdomen was negative. Musculoskeletal examination showed moderately decreased range of motion in the neck, shoulders, and elbows. The wrists were subluxed; the fingers showed ulnar deviation, subluxation of the metacarpophalangeal joints, and telescoping of the phalanges. The hips, knees, and ankles all showed decreased range of motion.
Fig. 10. Case 2. Rheumatoid arthritis. The lateral cervical spine demonstrates erosion of the odontoid and subluxation of the atlantoaxial joint. Rarely, erosions of the spinous processes occur as well; an example is seen at C5.
OPERA-GLASS
HAND
Fig. 11. Case 3. (V.L.) Rheumatoid arthritis. Generalized osteoporosis and severe interphalangeal joint destruction are present. The distal interphalangeal joints are narrowed and small erosions are present. Concentric resorption of the distal ulna and a pressure erosion of the proximal phalanx of the thumb add to the deformity.
No subcutaneous
nodules were present and neurologic
of the hands was normal, Bengal staining laboratory
11.9, WBC
4.3,
urinalysis
x ray was normal
and ECG
DNP
antibody
Case 4:
normal,
negative.
arthritis (V.R.)
keratitis.
changes.
antibody
rheumatoid
arthropathy.
rheumatoid
in 1935 at age 19. shortly
1968 she noticed syndrome mainly
marked
for many
of aspirin:
arthroplasty
The patient
rapidly
progressed
she has never had
in September,
fingers. subluxation phalangeal
joints,
after
50. albumin
normal.
Chest
was negative,
serum complement
anti-
normal.
and
changes compatible
with
termination
syndrome,
Her
hands
She has a history
treatment.
in
disease
and
since the onset. and from nodules.
re-
fusion
who had the onset of
of a pregnancy.
srlastic implants
interphalangeal
and focal
thumb
her feet. knees, and
of her hands. steroid
wrists,
Sjogren’s
then
1963 to
of Sjogren’s
Treatment
has consisted
She has had
a metatarsal
of the metacarpophalangeal
jornts of the right long and ring tingers,
fuston.
1968, showed: Musculoskeletal
contracture,
wrests had a total
of the metacarpophalangeal dislocation
factor
representative
ESR
enrymes
white female
to be active
parenteral
joints of both hands and of the proximal Examination
serum
destructive
She has never had subcutaneous
and a right thumb interphalangeal
are
normal,
successful interphalangeal
to involve
increase in the deformity
of the left foot, fusion of both
bows had a 20” flexion
negative,
is a 56-yr-old
disease has continued
years.
following
Rheumatoid
arthritis,
an area of resorptive
The
sensory examinatron
changes in the hands.
in the hands, with a subsequent
became generalized.
The 2.5,
showed
sorptive arthropathy arthritis
including
2 mm of wetness and the Rose
platelets
phosphatase
X rays of the joints
12) Seronegative
began in the left shoulder,
less than
differential),
alkaline
anti-DNA
with resorptive (Fig.
pun&ate
(normal
showed nonspecilic
positive undiluted,
LE cell preparation rheumatoid
4800
examination,
test showed
of the eyes demonstrated
tests: Hb.
3.8, globulin
The Schirmer’s
joints.
of the metacarpophalangeal
system: shoulders
of 20” motion,
flexion contractures and proximal
ulnar
abduct
to 80”. el-
deviation
of
of the proximal interphalangeal
all
inter-
joint
of
SWEZEY ET AL
Fig. 12. Case 4. Rheumatoid arthritis. Although subluxation of the metacarpophalangeal joints and the wrist are the most prominent abnormalities, there is resorption of the proximal phalanx of the thumb and subluxation of the distal articulating bone.
the right thumb and NalebufT 2 deformity of the left thumb, Rexion contractures of both hips and knees, No subcutaneous nodules were present. She had marked erythema about her fingernails and palmar surfaces. The rest of the examination including the neurological was normal. Schirmer test showed 0 and 2 mm of wetness. Laboratory tests: Hb. 13.0, WBC 8200 (normal differential), platelets normal, urinalysis normal, ESR 47, creatinine 0.9, albumin 3.5, globulin 4.5, rheumatoid factor negative, anti-DNP antibody positive in I:4 dilution, anti-DNA antibody negative, serum complement normal, LE cell preparation positive. Chest X ray showed linear densities at both changes with resorptive bases, probably old. X rays of her hands showed chronic destructive changes in the interphalangeal joints of the right thumb. In January 1970 she had an acute myocardial infarct with no previous history of heart disease. In December 1971, surgical bone fusion of the right interphalangeal joint of the thumb was successfully performed and this represents the only report to our knowledge of a surgical fusion in a patient with resorptive arthropathy.
Psoriatic
Arthritis
The propensity for psoriatic arthritis to lead to bone resorption has been rePsoriasis and typical rheumatoid arthritis coexist in peatedly observed. 1~2~7~10~‘7~32 psoriatic arthritis about 18% of psoriatic patients.33,34 Features distinguishing from rheumatoid arthritis are: the usual lack of rheumatoid factor and rheumatoid nodules; the greater prevalence of distal interphalangeal involvement; the greater tendency to asymmetry of joint involvement; and the increased tendency to bone proliferation in the psoriatics.10,32-36 Psoriatic skin lesions occur prior to or simultaneous with the onset of arthritis in the vast majority of patients. 32 Nail changes have been observed in 80”/,
OPERA-GLASS
HAND
207
Fig. 13. Case 5. Psoriatic arthritis. Both rheumatoid and psoriatic arthritis may cause severe resorptive changes in the hands. Several radiographic distinctions between the two diseases are illustrated. (1) Bony mineralization tends to be maintained. (21 Bony ankylosis of the interphalangeal joints is far more frequently seen in psoriatic arthritis. (31 Extensive involvement of the distal interphalangeal joints is present. (4) Widening of the joint space from excessive fibrous deposition is present at the fourth and fifth proximal interphalangeal joints.
of psoriatics who developed arthritis. 32 Psoriatic erythroderma has been reported to be more common in those patients developing psoriatic arthritis and was reported to occur in four of seven cases of resorptive arthropathy in one series.32 Peripheral joint ankylosis has been noted in almost half of the psoriatics developing resorptive arthropathy. 32 Sacroileitis has been noted in about i of patients with Reiter’s syndrome and the close relationship of these syndromes
Fig. 14. Case 5. Psoriatic arthritis. Aesorptive changes in the foot are most extensive at the pressure points and thus deform the metatarsophalangeal joints. Bony ankylosis of the proximal interphalengeel joints and severe erosion and widening of the distal interphalangeal joints distinguishes this pattern of destruction from the changes of rheumatoid arthritis. Deposition of fibrous tissue at the interphalangeal joint of the great toe with resultant widening of the joint space is a frequent finding in psoriatic arthritis.
208
SWEZEY
ET AL.
has been documented.34.37*38 Two of Green’s39 patients with psoriasis and severe erosive arthritis gave a history consistent with Reiter’s syndrome, and one patient with Reiter’s syndrome along with resorptive arthropathy has been reported .40 Case 5: (L.B.) (Figs. 13 and 14) Psoriasis with arthritis and resorption of the distal phalanges. The patient is a 49.yr-old white female who has a 33-yr history of psoriasis for which she has been treated with local steroid creams and parenteral steroids. She also has a 30-yr history of associated arthritis with involvement of the hands, feet, and neck with resultant prominent deformity in the hands and feet. Significant physical findings demonstrated psoriatic plaques over her back, buttocks, trunk. and extremities, pitting and grooving of the nails, and deformity of the hands with resorption of the distal phalanges (right greater than left) and shortening of the toes. She has no other joint involvement.
Ankylozing
Spondylitis
It is interesting to note that although sacroiliitis and resorptive arthropathy have been observed to coexist commonly in psoriatics and juvenile rheumatoid arthritics, evidence for coexistent ankylozing spondylitis or entereopathy with sacroiliitis and resorptive arthropathy is found in only seven cases.32~34~41~42 Zellner’s 5%yr-old male patient with severe psoriasis, generalized disease, and ankylosing of the cervical spine is presumably a case of ankylosing spondylitis.’ One of Soloman and Stecher’s patients, a 33-yr-old woman with juvenile rheumatoid arthritis, was described as having an ankylosed spine.” Two additional patients with ankylozing spondylitis, psoriasis, and “arthritis mutilans” are cited by Wilkinson and Bywaters, 43 but the only details mentioned are fusion of the metatarsal phalangeal and interphalangeal joints. The patient described by Friedman et al. was a 62-yr-old white male who had psoriasis, positive LE smears, and ankylosing spondylitis. ** Smyth described a 23-yr-old man with Still’s disease, resorptive arthropathy, and ankylosis of the spine, elbow, wrists, knees, and ankles.‘* A black female with ankylosing spondylitis and marked cystic lesions confined only to the humerus but no peripheral bony resorption, has been reported. I9 Marie and L&i’s patient was an elderly female and although no mention of spine radiographs is made, they describe her as having a complete ankylosis of the vertebral column. However, because of the relative rarity of ankylosing spondylitis in females, and the advanced age (70 yr) of their patient, it seems reasonable to postulate that the clinical appearance of ankylosis of her spine may have been attributable to osteoarthritis and contractures secondary to prolonged invalidism. Juvenile Rheumatoid
(JRA )
Resorptive arthropathy has been observed in three cases of juvenile rheumatoid arthritis.‘2~‘6~i7 The onset of arthritis in one case began at the age of five and the onset of the arthritis for the other two in the second decade.16 Aside from the resorptive arthropathy, no other unique features of the patients with juvenile rheumatoid arthritis have been noted.‘2~‘6~‘7 Case 6; (M.H.) (Fig. 15) Juvenile rheumatoid arthritis with resorptive arthropathy. The patient is a 24-yr-old female who had the onset of rheumatoid arthritis in 1952 at age five with pain and swelling in the ankles. This soon progressed to generalized joint involvement requiring steroid therapy during the first year of her disease: however, she developed severe cushinoid side effects and the steroids had to be discontinued. Over the next few years she developed progressive deformity of her wrists, hands. fingers, and elbows. At age six she had poliomyelitis which
OPERA-GLASS
HAND
Fig. 15. Case 6. Juvenile rheumatoid arthritis. Occasionally, severe destructive changes and erosions occur in juvenile rheumatoid arthritis and resorption of bone is extensive enough that overlapping of soft tissues causes an “opera glass” hand. Subluxation of the metacarpophalangeal joints and bony ankylosis of the wrist are additional stigmata of the juvenile onset of an inflammatory arthritis.
lel’t her
with residual
weakness in the left knee. At age 13 she had a supracondylar
the left knee and was treated with In
1966 she had a left Achilles
arthrodests.
The general activity
with chronic deformities fever. skin
rash.
nor
cutaneous nodules. microenathia.
without other
swelling
both
ESR
56,
is otherwise
platelets
of juvenile
rheumatoid
hands. Functionally.
ankle\
normal.
negative. 1.E cell preparation istic
thumbs
with
associated
at present she can stand for about
as an active college student
examtnation
13.3. WBC normal.
resorptive
I
9600
tests
although
in
both
bilaterally
contracture,
Joint
joints
is normal.
(normal
normal,
the arc
General
differential).
rheumatotd
factors
X rays were character-
changes rn the joints
of the
hr. can walk with crutches for one block,
She has moderate stiffness
write.
Joints
knee has a flexion
functton
negative, chest X ray and ECG arthritts
of both elbows,
and the metatarsophalangeal Hh
liver
suh-
1961 sholced.
appearance and havmg the feeling 01
the right
studies:
normal,
pain and is able to feed herself.
tain herself
triple
decreased motion
contractures
and metacarpophalangeal
have no motion
hut uses a wheelchair for most of her activities. has little
ankle
has never had in July
decreased mobility,
with a telescoping
Laboratory
urinalysis
She
Examination
nodules arc present and the neurological
normal,
of
of the knee.
her disease she did not have
disease.
asptrin.
with markedly
both hips have decreased motion.
swollen. No subcutaneous examination
of Still’s
been with
no motion,
the fingers are foreshortened ankylosed.
fracture
sttffness
and also a bilateral
or much pain. Throughout
and abduction to 45”. 20” flexion
with flexion
being very fratl.
procedure
characteristic
has mainly
wrests held in 25” of Rexion uith
left knee is
tn a residual
of her disease appeared to decrease in 1967, and she was left
lindings
Treatment
resulted
tendon lengthening
neck held in 30” Hexion
both shoulders suhluxed.
spica casts. This
and assist
in transfers.
recently
she is having
in the mornings She
hut
is able to main-
increasing
difficulty
in
moving her upper extremities.
Other
Collagen
S~~stenlic
Diseases
Lupu.~
Er~thrn~atosis
is characterLed
by a paucity
ceptions
are
to this
recognized
(SLE):
Systemic
of destructive and
so-called
articular
lupus
erlthematosis
involvement.‘“~”
crossover
cases
with
(S1.k.) hlanl
rheumatoid
ex-
210
SWEZEY
ET AL.
arthritis and other “collagen” diseases are well documented.46m4y Positive LE smears and antinuclear antibodies occur commonly in patients with rheumatoid arthritis, but these patients have a course typical of rheumatoid arthritis.50%56 It would, therefore, not be unreasonable to assume that a coexistent case of SLE and resorptive arthropathy would occur. None have so far been reported; however, we have observed one patient (W.S.) who demonstrates this combination in addition to Sjogren’s syndrome. Cuse 7: (W.S.) (Fig. 16) Systemic lupus erythematosis and rheumatoid arthritis with resorptive arthropathy. The patient is a 44-yr-old black female whose illness began in April 1951 with migratory arthralgia and a butterfly erythematous rash on her face. That same year she developed pieurnis and was diagnosed as having SLE. In June 1952 she presented with pleuropericarditis, butterfly rash, alopecia in the temporal region, and anemia. SLE was again diagnosed although no LE ceils were seen on bone marrow or peripheral blood smear. Laboratory studies of record showed: Hct 30, WBC 5,000. urinalysis normal. She was treated with aspirin. In 1952 she also developed pain and swelling in the joints of her hands and feet which progressed to severe deformtty over the next 10 yr. InHammatory involvement of the elbows, knees and ankles had been present but dtd not result in deformity. In 1956 she began complaining of dryness of the eyes and mouth persistmg to the present. Eye examination in April 1968 showed the presence of healed and vascularized cornea1 ulcers. Schirmer’s test in June 1970 showed wetness to 35 and 22 mm. There is no history of Raynaud’s phenomenon, subcutaneous nodules, dysphagia, scleroderma, or other skm changes. When first seen at the LAC/USC Medical Center in 1968, examination was unremarkable except for severe shortening of all the digits of her hands and the fourth and fifth toes of the right foot. The skin over the digits was loose and redundant. The involved digits demonstrated the classical telescoping appearance of the main en lorgnette deformity. All involved joints were painless. Examination of other joints was normal. Significant laboratory studies showed: Hb. 10.9, WBC 4,000, ESR 54, BUN6, normal urinalysis, SPEP normal except for a gamma globulin of 3.1, LE preparation positive, anti-DNA-protein antibodies I: 16, anti-DNA antibodies negattve, rheumatoid factor 1:80, serum complement 640 (normal). Chest X rays and pulmonary function tests were compatible with restrictive lung disease secondary to tibrosn. Esophageal X ray showed a dilated atonic esophagus. Renal function has always been wtthin normal hmits. Treatment since 1968 has been only aspirin. Significant past history includes a htstory of syphihs diagnosed and treated in 1951 with 43 million U pencillin. In 1970
I
Fig. 16. Case 7. Systemic lupus erythematosis and rheumatoid arthritis. Cartilage loss and resorptive changes are limited to the fingers and have caused severe loss of bone and redundancy of the soft tissues and the classic picture of an
OPERA-GLASS
the VDRL
211
HAND
and TPI were positive.
It was felt that
the pattent
had
received
adequate
therapy
for
her syphilis.
Progressive Systemic Sclerosis (PSSI: Like SLE, progressive systemic sclerosis (PSS or scleroderma) has been identified with a lack of invasive synofeature of subvitis or pannus. jjs9 The unique, but by no means constant of positive rheumatoid synovial fibrosis is peculiar to PSS.j7.‘” The association factors and a more aggressive synovitis has been reported.“,j’ Positive antinuclear antibodies and a close clinical relationship to SLE are also frequently Seen ,48.49X60,61
Distal digital resorption is a well-recognized feature of PSS.62,67 This has been attributed to ischemia associated with Raynaud’s phenomenon and the attritional effects of the sclerodermatous process in the skin overlying the phalanges.62,68 An apparently self-limiting form of Raynaud’s phenomenon and digital tuft resorption has been reported in men exposed to polyvinyl chloride.h9 An intriguing experiment of nature has been reported in which a patient had a traumatic amputation and successful reattachment of the distal half of her left fourth finger at age three and subsequently developed scleroderma at age 50. Raynaud’s phenomenon and resorption of the fingertips and distal phalanx occurred in all but the formerly amputated finger.” Although some sparing of a neurovascular defect by the amputation and reanastomosis is clear, the exact mechanism remains obscure. PSS, indistinguishable from rheumatoid arthritis. has been described in only two patients, and the evidence presented for scleroderma in one of these cases is incomplete. “Her fingers were cold, clammy. and cyanotic, suggesting sclerodactylia and Raynaud’s disease, for which bilateral sympathectomies were performed.“” The second case reported by Dubois et al.49 is again described in this report (Case 8) and probably represents an example of the coexistence of rheumatoid arthritis and PSS (see Fig. 16). Case 8: (D.T.) (Fig. 17) Progressive systemic sclerosis and rheumatoid arthritis with resorptive arthropathy. This patient is a 27-yr-old black female whose illness began at age I6 -81th prominent swelling and tenderness of the small joints of the hands, knees. and ankles. She was initially diagnosed as having rheumatoid arthritis because of an associated positive latex fixation test; however, over the next year she developed the typical skin changes of scleroderma Involving her face and hands. She also experienced intermittent Raynaud’s phenomenon. Skin biopsy at that time was read as compatible with scleroderma and an upper gastrointestinal X ray showed lack of esophageal peristalsis. Over the next 3-4 yr her arthritis progressed, generalized. and resulted in deformities and flexion contractures so that she was confined to a wheelchair. A major problem from the second year of her illness to the present has been recurrent skin ulcerations over the knees, lateral and medial ankles. soles of the feet, and tingertips. In October 1967 she suffered an autoamputation of her right fifth toe. In 1968 she complained of dryness of her eyes and a Schirmer’s test showed 9 mm of wetness in one eye and I mm in the other after 5 min. Examination of the cornea was normal. Musculoskeletal examination in October 1968 showed micrognathia. limitation of neck rotation, main en lorgnette changes in the hands, moderate flexion contractures in wrists. elbows, shoulders. knees, and ankles. Neurologic examination was normal, including sensory exammation of the hands Phystcal examination was otherwise normal. Significant laboratory studies tn August 1970 were’ Hb. 12.4. WBC 7900, ESR 47, normal urinalysis, BUN 10, normal serum enzymes. SPEP normal except for gamma globulin of 2.0. Representative LE preparations have been positive. anti-DNP antihodies positive undiluted, anti-DNA antibodies negative, and rheumatoid factor was 1:2,560 She has had no pulmonary disease clinically or on X ray and no leukopenia suggestive of a Felty’s syndrome Treatment over the years has consisted mainly of steroids (Prednisone up to 20 mgiday) and she is presently taking 5 mg/day.
SWEZEY
Fig.
17.
Case
progressive gressive
systemic
toid arthritis syndrome have
in
Fig. 18.
Case
are sule
present.
the frequency of the may
in which
proximal
be
calcium
occurrence
in the related deposit.
hand.
tissue
within when
the
the
of
sclerowasting
joint
cap-
compared
subcutaneous Erosion
phalanx to
systemic
and soft
Calcification
is a rare
are seen
9. Progressive
osteoporosis
collections
of the the
contiguous
with
distal
end
second
finger
soft
tissue
sclerosis
this
bone
as
as
well
well
as
as
of
and
of prorheuma-
to Sjogren’s
Resorptive
loss
ulna.
arthritis
Elements
in addition
patient.
marked
ance to the distal
sis. Diffuse
Rheumatoid sclerosis.
are present
caused
phalangeal
8.
systemic
ET AL.
changes
metacarpal
a whittled
and
appear-
OPERA-GLASS
(‘are
Y.-
(L.C.)
to represent white
HAND
(Fig.
18) Classic
an example
female
developed
213
who
had
arthritis
the onset
involving In
Increasing
sktn changes
had intermittent
skin
of
or pulmonary
ulcerations
Increasing involvement.
main
She has noticed
been mainly continued
aspirin because
Involvement ulcer\
and
pain
pain
although
of gastrointestinal
on her
side
abdominal.
lingertips
and
and neurological
metacarpophalangeal. digital right
tips.
mal.
Thcrc
elbow.
motion
Hand
X rays showed
In the left
proximal
of the proximal bowel
anti-DNP
X ra!
tionb of the left angle but normal
and
dilation
folate
positive
in
around
There
carotene
.joints.
Esophagus
X rays showed
colon.
vasculature.
Chest
bowel:
knees.
X ray ahoued
of
her
and
time
slight
nor-
50 300).
75”,,. rheumutold
deatructlw v,ith
changes ahoued
ol the
of the decreawd
platelets
antibody
changes
enema
se\cral
contracture
dill’erential).
classical
blunting
on
of her wrist\.
72 mcp IA0 (normal
mutllans
barium
h,l\
bc dis-
of hcalcd
present
resorption
and PIP joints,
arthritis
\mall
zre
antl-DNA
foreshortening.
IS the
sclerodermatou\ tvidence
flexlon
5 mg “,,. prothrombin ,yj dilution.
ankles.
do not clear on coughing.
90’
4600 (normal
feet,
had to
is ankylosts
and
jhe
Treatment
typical
marked
joints.
the Nrlsta
Four CYS.
deformit)
but
and hand\.
with
with
of the proximal
transverse
pulmonary
was trlcd
of the hip\ serum
knees,
months.
feet. Telangiectasia
joints
I I .5. WBC
14. K 4.2.
soft tissue calcification phalanges
few
shows
are negative.
motion
315), serum
antibody
interphalangeal showed
normal BUN
I35
Penicillamine
of her
01
She ha\
cardiovascular.
are the
the past
of her shoulder
teats: Hb.
normal,
82 mg “,, (normal
negative.
balls
ahc
because
body.
in these joints
E‘r;amination
Interphalangeal
IimitatIon
Laboratory
23. urinalysis
but
over the face. arms.
examinations
neck motion.
of the ankles. LSR
the
of renal.
have fine rales at the bates which
proximal
IS moderate
decreased
cholesterol factor
and
etTects.
very marked
was made
of her whole
joints
hands.
medication,
later.
to Involve
and over the past fete months
evidence
over
years
the halls of her feet for years.
floated
symptomatic and
of her ~a”
chest. neck. arid lower lips. The lung, Heart.
the maln
in the wrists
recedcnce
of the skin generally.
is prewnt
and under which
is ;I 52.!r-old
progressing
(PSS)
the skin
case appear\
patient
eventually
of scleroderma
She has no cllnlcal
At present,
She has minor
stools
This
The
at age 22. Fifteen but
involved
on her fingertips bulky
arthropathy.
arthropathy.
hands.
eventually
dysphagia.
and shoulders. problem.
and
1959. at ape 40. :L diagnosis which
resorptlve
phenomenon
the wrists
to live years ago she began noticing has experienced
and
resorptive
Raynaud’s
mainly
most of her joints. classical
scleroderma PSS with
of “pure”
right
negative. chungc\ telewwplng
for PSS: small pseudowcculacardiophrenic
ECG was normal.
Sjogren’.\ SJndrotne ISS/. It may appear somewhat artiticial to include Sjogren’s or the sicca syndrome as a separate form of resorptive arthropathy: however, it is included to emphasize the importance of its association with other disorders. The syndrome has long been recognized to occur with rheumatoid arthritis: however, its coexistence with PSS, SL.E, and polymyositis is less well appreciated.“~‘” ‘j Although Block et al.7J noted no difference in the severity ofjoint manifestations of patients with or without the sicca syndrome. live of our 35 patients with rheumatoid arthritis and the sicca syndrome observed prior to our study of resorptive arthropathq were found to have resorptive arthropathy, an incidence of resorptive arthropathy much higher than is expected in rheumatoid arthritis without the sicca syndrome.“.” It therefore seems doubly striking that the coexistence of Sjogren’s syndrome and resorptite arthropathy has been reported but twice. 2”,77Although Mather’s”’ patient had typical rheumatoid arthritis, the patient reported by Rogers and Lansbury” had the sicca syndrome associated with polychondritis. This is the only patient Mith the very rare polychondritis and resorptive arthropathy that we have been able to discover. Mulricentric
Rrticulohi.Ftioc??ro.Fis (Lil,oid-L)ertllrIfo-if
rthritisj
M ulticentric reticulohistiocytosis (M R) is a rare disorder single to numerous brown to yellow papular tnucocutaneous
characterized bq lesions hith ;I
214
SWEZEY
ET AL.
predilection for location on hands, arms, face, and neck.78m8”The lesions contain numerous large lipoid laden histiocytes which stain poorly with Sudan III and IV, but strongly positive with Sudan Black B and the periodic acid Schiff technique.7n The disorder may be confined to the skin, but it is often associated with a generalized acute and chronic destructive arthropathy in which synovial tissue has been shown to reveal the same histocytic changes.78~80 In a review of 23 cases, three-fourths occurred in women and essentially all ages and races have been represented.78,80~x2 One fifth of these patients had resorptive arthropathy.80,82 The distinguishing radiographic features of M R are more rapid bone than artitular destruction; extensive terminal interphalangeal involvement; and unusual sites of involvement including lumbar facet joints, cervical spine including the odontoid process, costotranverse joints as well as connective tissue attachments on the femoral trochanters, ischial tuberosities, anterior angles of vertebral bodies, and the shafts of Long bone such as the radius and ulna.7’~80~x3~84 The location of the skin nodules over the joints mimicking rheumatoid nodules may greatly add to the difficulty of distinguishing this entity. In addition to the radiographic features, the absence of rheumatoid factor, the not infrequent association of hypercholesterolemia and xanthelasma, and above all, a positive skin lesion or synovial biopsy serve to differentiate this unique disorder.78,7’ DIFFERENTIAL
DIAGNOSIS
Neuropathic Joints Although the neuropathic joint clinically and radiologically may be indistinguishable from a resorptive arthropathy, the neuropathic joint is invariably found in diseases that manifest impaired deep pain and proprioceptive sensations. The neuropathic or Charcot joint typically is characterized clinically by its remarkable instability, relative painlessness, and usually the presence of a marked effusion. Pathologically, with the exception of synovial chondromatosis, the presence of intrasynovial bone and cartilage fragments is unique.” Radiographically, the counterparts of clinically observed joint hypermobility, effusion, resorption of articular surfaces, fragmentation of articular bone, profuse disorganized hypertrophic articular and extraarticular bone proliferation are all features of the neuropathic joint.85 ” The two disorders most commonly associated with the Charcot joint are tertiary syphilis (tabes dorsalis) and diabetes mellitus (posterior column sclerosis and peripheral neuropathy).86,“8,89 Neuropathic joints are much less common in the upper extremity than in the lower, and when they occur in the upper extremities, they are usually found in 9om92 Tertiary syphilis, cervical ribs, association with leprosy and syringomyelia. and multiple sclerosis have been reported as causes of upper extremity neuropathic joints, and it is of interest that only one example of a Charcot joint occurring in the upper extremity (a wrist) has been reported with diabetes mellitus.” K9mY4 Charcot joints hdve been described in a number of other disorders including congenital insensitivity to pain, peripheral neuritis, spinal cord tumors, spinal
OPERA-GLASS
215
HAND
cord trauma or myelitis, malnutrition, and pernicious anemia with subacute combined degeneration of the spinal cord.95m98 The extremely rapid progression of some of the cases of Charcot joints (as little as nine days with associated sepsis and 446 wk in the absence of known coexistent infection) compares favorably with the most rapid recorded development of primary resorptive arthropathy, 4 mo as reported by Baker.32,87,92,94 Osteomyelitis is a frequent complication of the neuropathic joint.“‘~“‘.” It is particularly apt to occur in the fingers and in the metatarsal and phalangeal bones adjacent to the soles of the feet. 9o In syringomyelia and Hansen’s disease infection is frequently seen in association with resorption of the phalangeal tips of the hands.64 A number of other disorders recorded as producing similar digital tip resorption, although not necessarily on a neuropathic basis, include in addition to scleroderma, diabetes mellitus, syphilis, ainhum. bronchiectasis. Burger’s disease, hematoporphyria, polyvinyl-chloride exposure. and Ra!,naud’s disease.64m69 Thus, resorptive arthropathy can be distinguished clinically from neuropathic joint disease by the type of associated arthritic disorder, the lack of neurological manifestations and the lack of infection complicating the joint destruction. Pathologically, the lack of identifiable synovium in resorptive arthropathy contrasts with the intrasynovial bone and cartilage fragments that disiingulch the neuropathic joint.4.15.‘8.21,85 Casr 10:
(M.W.)
black female
with
(Fig. a 20-yr
19) Syringomyelia history
history of loss of temperature
and digital tuft resorption
of repeated
perception
burns and infections
in both hands and forearms.
The patient of both Over
is a 36.vr-old
hands and a Ii!-kr this permd
01 tune
her hands have become thick and fleshy. She has had a cellulitis of the right index finger in 1464. the left second and third fingers in 1964. the left hand in 1965. and the left middle finger I” 1967 Examination
in 1965 showed findings compatible
and a diagnosis compatible
Fig. 19. tissue langes, bone
Case
and
degenerative joint
are typical
disease.
10. Syringomyelia. resorption
of
the
arthritis
of the thumb of the changes
with a spinal cord lesion involving
was made. A myelogram
with a diagnosis of syringomyelia
swelling,
phalangeal
of syringobulbia
and
Diffuse distal of
the
ectopic
soft phainternew
in a neuropathic
was performed
C5- T9 or 10
and the findlnpa
were
216
SWEZEY
Fig.
20.
neuropathy foot.
Case has
Extensive
11.
normal severe a
of
the bony
joints toes
neuropathic
of bone and
condition from
Alcoholic
at
the
metadisloca-
Maintenance in the
arthropathy
picture
AL.
neuropathic
subsequent
present.
mineralization
resorptive
radiographic
is
foot.
in a typical
resorption
tarsophalangeal tion
Neuropathic
resulted
ET
presence
is characteristic and
distinguishes
rheumatoid
of of
a of
the
arthritis.
neuropathy, secondary osteomyelitis. and bone destrucCase II: (S.C.) (Fig. 20) Alcoholic tion. The patient is a 49.yr-old black male with a past history of chronic alcoholism with secondary peripheral neuropathy and a seizure disorder. He has a history of bilateral foot osteomyelitis dating back to 1941 with intermittent drainage from both feet over the years with variable Klebsiella, and Aerobacter organisms. He has no past history of positive cultures for Proteus, diabetes mellitus and a recent VDRL was negative. In 1963 his tifth toe was amputated due to the osteomyelitis. In November 1969 examination revealed draining sinuses in the left foot with stasis dermatitis, increased pigmentation. and swelling of the feet bilaterally. In April 1970 he developed a squamous cell carcinoma in the floor of his mouth. Case I2: (F.M.) (Fig. 21) Diabetic neuropathy, plantar ulceration, and secondary osteomyelitis. The patient is a 54-yr-old white male who had the onset of diabetes mellitus in 1950 at age 37. The diabetes required treatment with insulin (NPH-20-30 units per day) for the first 14
Fig.
21.
changes langeal
Case of
the
metatarsal osteomyelitis multilated
12.
Osteomyelitis.
metatarsal
bones
neuropathic ever,
the
are
and shaggy
similar psoriatic
periosteal
bones as extremity.
to
proximal the
pha-
changes
arthropathies. new
suggests the
Resorptive
and
underlying
the
bone
along
presence cause
of How-
of
the of this
OPERA-GLASS
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Fig. 22. Case 13. Leprosy. It is rare for a neuropathic condition to be unaccompanied by infection when it occurs in the foot. The resorptive changes that are seen therefore may be due either to the primary neuropathic problem or the coexistent infection. Plantar ulcers were present beneath the fourth and fifth proximal phalanges and appear to have accelerated the resorptive changes. Additional resorption is seen in the distal phalanges of the second, third, and fifth toes.
yr of the disease and was controlled with diet alone after that. He has a significant history of excessive alcohol intake since 1943, requiring numerous admissions to hospital for delirium tremens. In 1957 he complained of burning of the soles of his feet and on examination jn 1959 was found to have decreased sensation to pinprick in the legs from mid-thigh down to the toes. He has been noted to have decreased arterial pulses in his feet since 195X. In I960 he developed osteomyelitis in the left foot secondary to an infected plantar ulcer and in 1963 he burned the soles of both feet on hot asphalt and subsequently developed chronic plantar ulcerations on both feet with btlateral osteomyelitis. In April 1967 he had bilateral above-ankle amputations as treatment ol osteomyelitis. Pathologic examination of the feet showed arteriosclerosis obliterans changes III the blood vessels. Of significance in the past history is intermittent hypertension since 1940. HIS father, brother, and sister have diabetes mellitus. Significant physical flndings other than the findings in the legs were mild hypertensive arteriolar findings m the vessels of the eyes. Lahoratory tests show elevated blood sugar, evidence of liver disease (BSP 26”,, at 30 min). and nonspecific ST changes on ECG. Case 13: (M.S.) (Fig. 22) Leprosy with neuropathy. The diagnosis of leprosy was made in thts 35.yr-old white female in 1961. She presented symptoms of decreased sensation and joint swelltng in the lower extremities. Examination demonstrated many ulcerations over the legs, a spotty dccreased sensation over the calves. decreased sensation over the area of the distal ulna with atrophy of the hypothenar eminence of the left hand, swelling of the distal phalanges of both fifth fingers, and hepatosplenomegaly. Liver biopsy showed multiple granulomata without necrosis or giant cells. Acid fast organisms were demonstrated. Skin biopsy &as characteristic of lepromatous leprosy
Erosive Osteoarthritis
Erosive osteoarthritis (EOA) is the most recent arthropathy to be identified in the group of disorders associated with resorptive arthropathy. In two separate papers in 1966, Peter et al.‘“‘~“” called attention to a particularly aggressive, destructive form of primary osteoarthritis, which by virtue of an inflammatory synovitis caused marked erosion of distal and proximal interphalangeal joints. One of the patients (Case 5) reported by Peter, Pearson, and Marmor’“O demonstrated destructive changes compatible with resorptive arthropathy. but none of these cases manifested the resorption of the adjacent bone. C‘UW14: (R-B.) (Fig. 23) Erosive arthritis with severe distal and proximal interphalangeal
SWEZEY
ET AL.
Fig. 23. Case 14. Erosive osteoarthritis. The typical distribution of changes in erosive osteoarthritis are present in both hands, involving the distal and proximal interphalangeal joints, and the trapeziometacarpal areas. The erosions and subluxations are so extensive that a wavy contour of the fingers mimics the appearance of an “opera glass” hand.
joint destruction. The patient is a 61-yr-old white housewife with progressive distal interphalangeal and proximal interphalangeal arthritis of at least 5 yr duration, Aside from trapezoimetacarpal hyperthrophic changes, no other joints were involved. There was no prior history of arthritis. There was no evidence of psoriasis or systemic disease. No intraarticular steroids bad been administered. rheumatoid factor
Sensation and determinations,
motor and
function serum
were intact. The CBC, sedimentation uric acid were all within normal
rate, limits.
The hands demonstrated marked digital deformity with hypermobile interphalangeal joints and some redundancy of overlying skin. There was only slight tenderness of the affected joints. Radiographs demonstrated extensive distal and proximal interphalangeal joint erosion and subluxations with hypertrophic changes in the trapeziometacarpal joints. This case is presented as an example of erosive osteoarthritis and severe joint destruction without actual bone shaft resorption.
A vascular Necrosis
Avascular necrosis occurs insidiously in the absence of any associated arthritis. It is extremely rare in the small joints of the hands. It is best distinguished from resorptive arthropathy radiographically early in the course by the lack of joint margin erosion and by the destructive changes limited to only one articular surface. Later when both joint surfaces are involved, the joint space is narrow and secondary proliferative and degenerative changes predominate. Avascular necrosis of bone occurs when an area of bone has been deprived of its blood supply by any means, excluding infection. The areas most frequently involved are those with a tenuous blood supply and poor collateral circulation, such as occur in the epiphyseal areas. In the adult, common epiphyseal locations affected are the head of the femur, the humeral head, distal femur, and proximal tibia. Less common locations are the distal tibia, body of the talus, carpal scaphoid and lunate (common after trauma), and the humeral involved. In the juvenile capitellum. lo3Two or more areas can be simultaneously with a developing skeleton, other areas involved are the vertebral bodies, tibia1 tuberosity, patella, OS calcis, and head of the metatarsal.“* Shawl” described a case involving the phalanges of the hands (Thiemann’s disease); however, a destructive arthritis did not result. The earliest X-ray finding is an area of arclike radiolucency paralleling the (a relative or subchondral surface of the involved bone. lo4 Later, osteosclerosis absolute increase in the X-ray density) of the affected bone end occurs and
OPERA-GLASS
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cystic and sclerotic islands appear in the resulting, often wedge shaped, infarct of bone.“” Continued stress and weight bearing produces secondary subchondral fractures in the necrotic and newly formed bone resulting in fragmentation and collapse of the bone with irregularity of the joint margin, destruction of the cartilage, and prominent secondary degenerative joint changes. Avascular necrosis has been reported to occur in association with many disease states. They include hemoglobinopathies (mainly sickle cell disease and sickle cell trait, hemoglobin SC disease, and hemoglobin S-Thal disease). caisson disease, dislocations and fractures of the hip, SLE, giant cell arteritis, pancreatitis, alcoholism, Gaucher’s disease, Cushing’s disease, steroid usage (systemic or intra-articular). hyperuricemia and gout, and renal transplantation (probably due to associated steroid treatment).‘“’ ‘I6 Although the exact mechanism of production of avascular necrosis by all of the foregoing is not known, the end result is the obliteration of the blood supply to a particular area of bone. Avascular necrosis does not commonly produce a picture one would confuse with resorptive arthropathy. Typically, it is the weight-bearing joints that are involved, and from a review of the literature, it appears that when multiple joints, including non-weight-bearing joints are involved patients are usually on high steroid dosages, e.g., renal transplantation patients.“b One such case had a total of 10 separate joints involved.“’ Uncommonly, the small bones of the hand are involved and in those cases, resorption is not a prominent hnding.“h The case we have presented (A.S.) is unusual in the number of joints involved and the severe destructive process incurred.
Fig. 24. Case 15. Avascular necrosis. Chronic high dosage of adrenocorticosteroids in a patient with systemic lupus erythematosus has resulted in avascular necrosis of both shoulders and hips. Loss of the humeral heed and diffuse osteoporosis are present.
SWEZEY
ET
AL.
Fig. 25. Case 15. Avascular necrosis. Similar changes of the femur have caused marked resorption of the femoral head.
Case 15: (AS.) (Figs. 24 and 25) Avascular necrosis of multiple joints in a patient with systemic lupus erythematosis on high dosages of steroids. The patient is a 35-yr-old white female whose history dates back to 1962 at which time she had the onset of polyarthritis associated with pneumonitis, anemia, and hair loss. A diagnosis of SLE was made on the basis of positive LE cell preparation and compatible clinical findings and she was started in high dose steroid therapy, at times as high as 200 mg/day. She was on a very high dose of steroids for several months at which time she was first seen in our clinic. She was gradually tapered to a maintenance dose of 4-8 mg methylprednisone a day. Over the years she has had no severe manifestations of her disease except for intermittent increases in arthralgias. There has never been any evidence of renal disease. During a pregnancy in 1964 she began complaining of increasing pain in her shoulders, hips, and knees. and over the ensuing months, a diagnosis of avascular necrosis in each of these joints was made. In April 1970 she developed a Staphylococcus mucus infection in her right hip joint. Later, pseudomonas organisms were cultured from the same area. In August 1970, a Girdlestone operation was performed on the right hip. However, she then developed difficulty voiding and woke associated one morning with a tingling sensation from her waist down to her toes bilaterally, with increased weakness and numbness in her lower extremities. An emergency laminectomy from T5 to T9 was performed, and she was found to have a collapsed seventh dorsal vertebra, presumably the result of severe osteoporosis, which was compressing the spinal cord. Postoperatively, she very slowly regained strength and sensation in her legs; however, recovery was not complete. Past history includes a bleeding duodenal ulcer in 1964 and 1969 and a left tibia] osteotomy in November 1969. A cousin has rheumatoid arthritis and an uncle may have scleroderma. On physical examination she is a chronically ill appearing woman. On examination of her heart, she has grade II/VI systolic murmur at the base without radiation. Pertinent physical findings are otherwise limited to her musculoskeletal system. The shoulders are limited to 90” and she lacks IO”--15” extension on the left. The right hip has the residuals of the Girdlestone procedure. The right knee has normal motion, but the left knee is posteriorly subluxed and has a range of motion from 25” I IO” with significant pain on motion. She is incapable of ambulation. In
OPERA-GLASS
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January 1972, on examination, she had generalized muscle weakness, more pronounced in the lower extremities; however, sensory examination and tendon reflexes had returned to normal. Laboratory tests: Hb. 8.5, WBC 11,000 (normal differential), platelets normal, urinalysis showed many WBCs without significant bacteriuria. She has never had significant RBCs or casts in the urine and 24-hr urine studies have never shown significant proteinuria. Other test results are: BUN 20, LE cell preparation positive, anti-DNP antibody positive undiluted, anti-DNA antibody negative serum complement normal, rheumatoid factor negative, ECG normal, chest X ray normal.
Sickle
Cell Disease
Sickle cell disease in the adult is characterized by avascular necrosis and large joint involvement. Osteomyelitis is not an uncommon complication in both children and adults. In young children, hands and feet may be involved in a disorder distinguished from resorptive arthropathy by the presence of periosteal proliferation, and occurring in an age group in which resorptive arthropathy has not been seen. Osseous involvement in sickle cell disease is estimated to occur in up to 65”,, of patients.“’ Changes have been reported not only in homozygous SS disease but also in SA, SC, and S thalassemia.“8 The basic pathogenesis in all cases is vascular occlusion secondary to the sickling phenomenon initiated by deoxygenation of the blood. Those areas of the skeleton with the poorest primary and collateral circulation are predisposed to the most severe and frequent involvement. Skeletal involvement associated with sickle cell phenomena can be divided into the following three groups. This is the most common bony manifestation of (1) Hand-Foot Syndrome: ‘I9 in a series of 1200 patients in the disease in children.“’ Lambolle-Legrand, Africa, states that over 80”/, of the patients exhibited the hand-foot syndrome during their infancy and childhood. The condition occurs in repeated attacks from age 6 to 24 and is very rare past the age of 6 yr.‘18 Prior to the sixth year, the small bones of the hands and feet contain red marrow. After the age of 6 yr, the red marrow recedes from the small bones leaving less vascularity and less opportunity for sickling. Clinically, the affected children have swelling and pain of the hands, feet or both. The swelling and pain last up to 2 wk and then subside. The hands are frequently symmetrically involved and occasionally all four extremities are involved at the same time. X-ray findings show diaphyseal and metaphyseal periosteal elevation, subperiosteal new bone formation, and a moth-eaten appearance to the shaft.12’ The joints are not directly involved and usually all the bone changes are completely reversible.‘i8 Occasionally the bone changes are very destructive and Watson et al.“’ reported the complete disappearance of a metatarsal. (2) Avascular Necrosis: Golding et al.“* report a lO”/,)- 12:;) incidence of avascular necrosis in the adult. Hemoglobin SC was involved more commonly than hemoglobin SS disease. The findings are as described in a previous section on avascular necrosis. No cases with a marked resorptive quality have been reported. Osteomyelitis is a relatively frequent complication of hemo(3) Infection: globin SS disease. The most common infecting organism is Salmonella, although involvement by Staphylococcus, Streptococcus, Shigella, and E. coli have been reported.‘23,‘2’ It occurs more frequently in children thus making the
222
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differentiation from the hand -foot syndrome very difficult. Constant et al.‘23 reported a Salmonella osteomyelitis with total destruction of a proximal phalynx of a thumb and a middle phalynx of a finger. Rubin et al.‘24 reported a case of Shigella osteomyelitis with extensive destruction of metacarpals, metatarsals, and phalanges in a 23-yr-old patient. The osteomyelitis rarely involves the joint space and thus a very destructive bony process may be present without the joint features characteristic of a resorptive arthropathy. Steroid
Arthropathy
In addition to avascular necrosis induced by systemic steroid therapy, a Charcot-like arthropathy has been reported from the use of intraarticular steroids.‘25-‘29 These changes have been observed thus far primarily in the knees and hips with both local and systemic steroid therapy.‘25m’30 In only one patient (a 43-yr-old woman with osteoarthritis who allegedly received a minimum of 200 injections into each interphalangeal joint in both hands over a 3-yr period) has a steroid related resorptive arthropathy in interphalangeal joints been reported, and the possibility remains that this patient may have had erosive osteoarthritis.‘30 In most cases, pain relief with steroid therapy appeared to permit joint abuse and joint destruction.‘25 ‘29 The experimental demonstration of cartilage damage in rabbits receiving two or more weekly intraarticular knee injections supports the local destructive propensity of the steroid crystals.13’ Differentiation of this complication of steroid therapy from resorptive arthritis should not pose a significant problem because of the restriction of the steroid induced arthropathy to those joints receiving multiple injections. Further, resorption with remodeling of the shafts of the bones adjacent to the joints has not been seen. Gout
Chronic tophaceous gout may result in a mutilating arthritis, with erosion of bone from adjacent soft tissue masses. In addition to pressure erosions, intraosseous tophaceous deposits contribute to the bony destruction.‘32 Occasionally, avascular necrosis contributes to the dissolution of bone.‘i5 Distinction from the classic forms or resorptive arthropathies is evident both by the appearance of the soft tissue and the character of the erosions. In chronic tophaceous gout, soft tissue masses deform the soft tissues but do not cause a redundancy of skin. They may contain calcium.‘32*‘33 Their contiguity to the bony erosions is often apparent. Erosions tend to be periarticular and have a characteristic overhanging edge and sclerotic border, in contrast to the whittled resorption and the absence of productive new bone of the resorptive ar‘34Progression of the periarticular erosion to complete destruction thropathies. of the joint may occur and replacement of the cartilage by fibrous tissue may mimic the changes described in the classic main en lorgnette hand and foot. In chronic tophaceous gout, multiple joints are frequently involved and at times the radiographic picture is indistinguishable from rheumatoid arthritis.13* However, the similarity is in the well-circumscribed cystic changes of the hands and feet, and gout never mimics the bone shaft remodeling of resorptive arthropathy.
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Fig. 26. Case 16. Gout. Mutilating arthritis from chronic tophaceous gout may cause severe destruction of the adjacent bones. This is most evident at the distal ulna and a large erosion with an associated tophus is present. Chronic draining tophi may ultimately lead to amputation as seen in the fifth finger, contributing to the mutilating effect of gout.
Recently, an enzyme defect as etiologic factor of hyperuricemia has been discovered and described clinically as the Lesch-Nyhan syndrome. The syndrome incorporates hyperuricemia, occasionally acute gout, mental deficiency, choreoathetosis, and a propensity for self-mutilation, such as chewing off the finger tips. Primary destructive joint disease has not been described although tophi can be present.13’ C‘ase16: (F.M.) (Fig, 26) Severe generalized tophaceous gout with marked destructive changes simulating resorptive arthropathy. The patient is a 25-yr-old white male who has had acute attacks of pain, heat, redness, and swelling of the joints, recurring at intervals since age 13. The attacks have occurred in almost every joint in the body. He had repeated admissions for excisions of tophi. amputation of toes, and later a lower leg amputation. He was generally stiff and had very painful joints throughout the body. His spine was nearly ankylozed. Numerous tophi, notably along the anterior tibia1 and forearm regions were present. Blood pressure 180/124, LAB. NPN 20 mg%. uric acid 0.6”/,-12.4 mg% urinary uric acid excretion-over 2 g/day: PSP test--60”/” excretion in I hr; S.G. 1.016; Hinton negative. Radiographs revealed extensive soft tissue tophi and generalized joint destruction secondary to tophaceous deposits. Sarcoidosis
Sarcoidosis not uncommonly affects the bones and joints, each being involved in as many as 1.5):;,-207,, of cases. The arthritis produced by sarcoidosis may be acute (and often associated with erythema nodosum) or chronic. Neither type commonly results in joint destruction.‘37 ‘39 Bone involvement is seen radiographically as a diffuse osteoporosis, a resorptive whittling, a lacelike coarse trabecular pattern, or as punched out cystic lesions.‘40 Joint destruction has been reported to occur as an extension of the cystic lesions from the bone into the ankle joint and the authors have observed such a case involving the knee joint.‘4’m’42 Neither case could be confused with a resorptive arthropathy. Case IX demonstrates very extensive bone involvement by sarcoidosis in the hands: however, the joints are not involved. Case 17: (M.G.) (Fig. 27) Sarcoidosis with cystic and resorptive changes. The patient is an 8O-y/r-old black female in whom the diagnosis of sarcoidosis was established at age 55. Initially,
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Fig. 27. Case 17. Sarcoid. Although relatively rare, resorption of bone in patients with sarcoidosis results in a whittled appearance of the phalanges as is seen in the third finger, in addition to the more typical defects in the phalanges of the third and fourth fingers. The coarsened trabecular pattern of the middle phalanx of the second finger is the third bone pattern seen with sarcoid and implies diffuse involvement by noncavitating granulomas.
involvement was mainly cutaneous. At one time a severe lesion in the nasal septum necessitated the use of Prednisone, 20 mg/day. Bilateral hilar adenopathy has been present since the onset: however, she has had no other parenchymal involvement. Progressive involvement of the bones of her fingers and wrists are illustrated. In January 1971 she developed nodules on her vocal cords shown to be sarcoidosis on biopsy. Skin biopsies have all been consistent with sarcoidosis. Case 18: (I.H.) (Fig. 28) Sarcoidosis with marked cystic and lacelike destructive changes in the phalanges. The patient is a 42-yr-old black female who was first seen at age 39 because of painful hands. This problem became progressive, and she was observed to have severe destructive changes in her phalanges. Her chest radiograph demonstrated prominent hilar adenopathy without other abnormalities. Her PPD #I was negative and her serum uric acid normal. Details beyond this summary are not available as her record has been lost and the patient has not been seen since 1955.
Tuberculosis
Tuberculosis only rarely involves multiple small bones, and when secondary joint destruction occurs, at most a few joints are affected. Like sickle cell
Fig. 28. Case 18. Sarcoid. Diffuse involvement of the spongiosa may occasionally destroy the bone so extensively as to cause a radiographic picture simulating resorptive arthropathy. However, this is easily distinguished from the classic causes of resorptive arthropathies by the location of involvement. The process has destroyed the diaphyses of the middle and distal phalanges, but spared the articulating ends of the bones so that the joints are relatively normal.
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disease, the age group in which hands or feet may be diffusely involved is much younger than that of resorptive arthropathy which is a postadolescent disorder. Skeletal tuberculosis is probably the most common form of extrapulmonary tuberculosis today. Of 100 patients seen at the State University of Iowa Hospitals with extrapulmonary tuberculosis from 195551966, 35 had skeletal involvement, 20 lymphatic, and 19 renal. ‘43 The basic lesion in skeletal involvement is usually a combination of osteomyelitis and arthritis and in adults the weightbearing joints are most commonly involved. Davidson and Horowitz’43 give the following incidence of involvement: spine 50%; hip 15%; knee 1.5%; wrist, elbow, shoulder, other bones 20”;,. The juvenile age group has a much higher incidence of involvement of the small bones of the hands and feet and more commonly has multiple bone involvement.‘44 Tuberculous bone involvement can be divided into localized and disseminated forms. The localized form is a combination of osteomyelitis and septic arthritis and usually involves only one joint, but more rarely a series of joints (as in tuberculous vertebral osteomyelitis) may be involved. With chronicity the infection can result in marked bone destruction and restriction of joint motion.‘45 Kelley and Karlson’4h reported destructive processes involving the wrist and ankle joints, and others have reported involvement of the small bones and joints of the hands and feet with associated destruction of a phalynx or metacarpal.‘47~‘49 Although the cases reported have destructive changes, they are usually localized to one or two joints and do not have the diffuse resorptive quality of the classic resorptive arthropathies. The disseminated form of osseous tuberculosis is uncommon and occurs mainly in young children and young adults.‘jO Typically in tuberculous dactylitis, the peripheral tubular bones are expanded and involved with numerous cystlike lesions.‘s’ The joints themselves are rarely involved and thus this entity should be readily excluded in the ditferential diagnosis of a resorptive arthropathy. Recently the atypical mycobacteria have been receiving more interest, and joint involvement due to one of these organisms has been reported. Klienenberg et al.“’ reported a case of destructive arthritis involving the proximal and distal interphalangeal joints apparently due to a photochromogen. None of these joints in their case demonstrated the classic resorptive arthropathic changes.
Extensive resorption of joint surfaces is not a factor of hyperparathyroidism. The primary lesion of hyperparathryoidism is found in the bone. The incidence of bone changes on X ray in patients with hyperparathyroidism ranges from 20”,, to 30% in various series.‘j3,‘j4 The findings include subperiosteal resorption, osteoporosis, disappearance of the lamina dura about the teeth, resorption of the distal ends of the clavicles, loss of height and biconcavity of the vertebra, and the late findings of bone cysts and brown tumors. Actual joint involvement in hyperparathyroidism can occur by the following mechanisms: ( I ) Calcium pyrophosphate dihydrate (CPPD) crystal deposition disease,‘s’,‘5h (2) gout,‘j7.‘5K (3) periarticular calcification with inflammation,‘5y and (4) a condition described by Bywaters I60 in 1963 which can mimic rheumatoid
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Fig. 29. Case 19. Hyperparathyroidism. Extensive resorption of bone in hyperparathyroidism may rarely cause shortening of the phalanges and collapse of the overlying soft tissues. resulting in a clubbed shape to the distal fingers. The typical subperiosteal resorption alters the sharp outer contour of the bone in contrast to the changes of resorptive arthropathies. As in sarcoidosis, the joints are spared and the diaphyses are destroyed, further distinguishing the radiographic picture from the connective tissue disorders.
arthritis with joint inflammation and actual erosions in the joints on X ray. Bywaters believes the erosions are due to subchondral bone microfractures which result in dissolution of the hyaline cartilage. Case 6 in his series developed a complete collapse of the first metacarpal presumably from this process. Although erosions and minor resorption of the joints do occur, there have been no reported cases in the literature to date that could be confused with a resorptive arthropathy. The case of resorptive arthropathy reported by Nelson in 1938 was associated with a parathyroid adenoma, however, there was no clinical evidence of hyperparathyroidism.” Case 19: (C.J.) (Fig. 29) Primary hyperparathyroidism, secondary renal failure, and severe bone resorption. The patient was a 56-yr-old white male when he first presented to the LAC/USC Medical Center in June 1962 with azotemia. In 1946 he passed a single renal stone. He had a several-year history of polydypsia and polyuria; a 445-yr history of arthritis, specifically involving the left ankle and which had been diagnosed but not documented as gout in the past; general aching in the extremities for years; clubbing of the distal phalanges for years; shortening of stature and kyphosis; and the onset of nausea, vomiting, and uremia in May 1962. In June 1962 he had a calcium of 10.0, phosphorus of 4.7, and X rays of the bones were consistent with far advanced hyperparathyroidism. The patient’s uremia progressed and on November 8, 1962 surgery was performed and a parathyroid adenoma removed. The other three parathyroid glands were atrophic. Preoperative laboratory tests were: Hb. 9.7, WBC 7700, ESR 64, Ca 10.4, Ph 6.9, uric acid 10.8, Creatine 6.2, K 3.5, Na 137. Post-operatively, he developed hypocalcemic tetany. He died on February 4, 1963, of progressive uremia.
Biliary Cirrhosis
A destructive process involving the joints similar to hyperparathyroidism has been described in biliary cirrhosis with hypercholesterolemia.‘6’ The erosions in these cases were not severe and resorption was not present. In both of these diseases the mechanism of erosion is extra-articular in contrast to the primary intra-articular process of a resorptive arthropathy. Osteolysis
Although massive bone resorption characterizes the conditions that cause osteolysis, absence of primary joint involvement serves to distinguish these entities from resorptive arthropathy. Hemangioma: There are two major forms of osteolysis. The most dramatic
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dramatic form, massive osteolysis, was characterized by Gorham16* as a disorder primarily affecting children or young adults caused by a “benign” hemangioma. Multiple contiguous bones may be progressively involved, but the disease is usually self-limiting. ‘62m’b4The pelvic and shoulder girdles are the usual sites of involvement. Typically endosteal new bone is laid down as the periosteal cortex adjacent to the angiomatoses is resorbed. This gives the appearance of concentric shrinkage and tapering of the affected bone until the two cortices meet, merge, and disappear.‘64 Familial Osteolysis: Familial osteolysis comprises the second category and consists of adult and juvenile forms. Acre-osteolysis is a dominant disorder of adult life characterized by disintegration of bone of the phalangeal tips. but also involving metacarpals and metatarsals in a more inflammatory necrotizing osteolysis. Sensory changes are absent, but joint hypermobility and congenital anomalies of the skull and spine are frequently seen and secondary osteomyelitis of the phalanges is a common complication.“’ Essential osteolysis with nephropathy is another dominantly inherited disorder affecting children at age 3 and 4 yr with an active arthritis typically involving the carpals and tarsals. ‘b6,‘b7The process becomes quiescent and then late in the third decade, terminates with collapse and lysis of the affected bones and their partial extrusion through the skin over the volar surfaces of the involved joints. In some patients, the process is limited to one extremity and in others, elbows and a clavicle have been involved.‘6h,‘67 In about half of the patients, there is an associated slowly progressive glomerulonephritis with hypertension and ultimately death from renal failure.“’ Biopsies of synovia have revealed only proliferation and hypertrophy of smooth muscle cells in arterioles and some areas of dense collagen tissue and lakes of metachromatic staining. “’ Bone biopsy revealed areas of cortical bone. Four renal biopsies showed eosinophilic tubular precipitates while one of these showed moderate arteriolar sclerosis. ‘66 The pathogenesis of the resorptive phenomenon cannot be elucidated from these findings. The familial nature of this disorder and the lack of involvement of the interphalangeal joints serves to readily distinguish it from resorptive arthropathy. A self-limited osteolysis following trauma has been reported.‘6x One case involved a wrist, and the second, a distal clavicle. No biopsies were available from these cases, and it is therefore impossible to classify them further.lhx Werner’s
Syndrome
Werner’s syndrome is a Mendelian recessive disorder characterized by an adult onset of premature aging. ‘69+‘72Subcutaneous calcification, sclerodactyly associated with atrophic skin, and resorption of the distal phalanges cause this rare entity to be confused with progressive systemic sclerosis. The predominance of skin and skeletal involvement in the feet and relative sparing of the upper extremities should suggest a separate entity as this pattern of skin and extremity involvement is the reverse of scleroderma. Keratomas and indolent ulcers are commonly present on the soles of the feet, and skin ulceration with secondary osteomyelitis of the adjacent bone complicates the picture of resorptive changes.lhy ‘72
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Additional features of Werner’s syndrome differentiate it from scleroderma: premature aging with early cessation of menstruation in the female; arrest of growth at puberty; premature balding; juvenile cataracts; hypogonadism; osteoporosis; osteoarthritis; arteriosclerosis; and diabetes. Clinically, the patient has a birdlike face with beaking of the nose and loss of the periorbital connective tissue, short stature with spindly extremities and a weak, high-pitched, squeaky voice. Because these patients have a high incidence of malignant disease with a special predilection for the development of sarcomas, their distinction from scleroderma is important.‘69~‘72 Case 20: syndrome with severe bone demineralization and degenera(E.J.) (Fig. 30) Werner’s tive changes in joints and soft tissue in the feet. The patient is a 51.yr-old black female admitted to the LAC/USC Medical Center in October 1968 for evaluation of an ulcer in her right ankle, associated with pain and swelling of one month’s duration. A biopsy of this lesion revealed osteogenic sarcoma. She was seen by the Rheumatology Service because of intermittent pain and swelling of her wrists, metacarpophalangeal, proximal interphalangeal, knee, and ankle joints. Her hands and feet were the most severly involved. She had noticed these symptoms since 1962, and was bedridden for 2 mo in 1963. She had also noted thinning and graying of her hair for I2 yr, and cataracts of both eyes for many years. In the preceding 4 mo she developed anorexia, weight loss, and an exacerbation of her joint symptoms which had been quiescent since 1963. There is no family history of consanguinity. Her mother has diabetes mellitus, but her father is leg ulcers, and thinning of healthy. She has one brother with cataracts, small stature, diabetes, his skin over his lower extremities. Her sister and child are alive and well.
Past history revealed the patient to be gravida (toes were “shriveling up”) in 1957. right cataract repair in 1961. The patient was an alert female who appeared
2, para 2. She had a bilateral removal in 1959, and a right much
older
than
her stated
sympathectomy patellar tendon
age; blood
pressure
160/70, pulse 84 and regular, R 16, weight 100 Ibs. Her hair was fine, sparse and gray. There were scanty eyebrows and eyelashes. There was a beaked nose, recessed chin, and a small mouth. There was an aphakia-O.D. and a cataract-O.S. The fundi could not be visualized. There was a generalized lack of subcutaneous fat. The skin had punctate, dark brown lesions which did not blanch and some areas of hyper- and hypopigmentation. There was a Grade II ejection systolic murmur heard best at the base along the left sternal
Fig. 30. Case 20. Werner’s syndrome. Soft tissue calcification, resorption of the small bones of the hands, and subluxation of the distal phalanges have resulted in a picture of distal joint mutilation.
OPERA-GLASS
HAND
229
border. Neck. lungs, and abdomen were within normal limits. Peripheral pulses were normal. There was marked muscular wasting proximally and distally and firm masses were palpable in both triceps tendons and the olecranon bursa. Significant joint findings were: ulnar deviation; flexion contractures of the elbows, knees, and hips; marked atrophy of the skin over the lower extremities with marked atrophy and deformities of her toes; multiple areas of callous formation over the plantar surface of the feet; and a large swollen right ankle with ulcerating lesions. Neurologic examination was within normal limits. Hgb. 10.4, WBC 5700 (57-O. 28-14-I-10) with adequate platelets, slight anisocytosis and rouleaux formation, BUN 14, K 4.0, Na 132, Co2 28. PBI 3.5. T, test 12.7”,,, calcium 9.5. Pod 3.3. The alkaline phosphatase was 6.5, uric acid 6.4 mg %, total serum iron 25 pg %, IBC 243, and saturation lo”,,. Her GTT was abnormal (FBS 155 mg “4, I hr--185, 2 hr--193, 3 hr-I70 mg yO). serum complement 960 (normal), ANA negative, latex fixation negative, LE prep negative, antithymoglobulin titer l:l6. Serum proteins-A/G 3.8/5.2 with 1.6 beta and 2.3 gamma globulins. Radiographs revealed multiple soft tissue and vascular calcifications, degenerative joint disease in her hands and feet with severe demineralization in the bones of the feet, The right ankle revealed changes compatible with an osteogenic sarcoma. A bone and chest film were normal.
Involvement of multiple small joints in the hands by benign or malignant tumors has only been seen with multiple enchondromata. Although it is extremely rare for tumors to involve the bones of the hands and feet, this involvement does occur and can be classified as follows: (1) Soft tissue tumors. These include tumors of the skin, fat, blood vessels, lymphatics, nerves, and nerve sheaths. Usually they affect the bone only by compression, however, the more malignant tumors such as malignant hemangiosarcoma will occasionally metastasize to the bone producing destructive lesions. They usually do not involve the joints unless the tumor is highly destructive. 173Synovial sarcoma is a rare tumor which, although it does not usually arise from within the joint cavity, is capable of metastasizing to the joint and eroding it. ‘74 (2) Primary bone tumors. The solitary enchondroma, osteochondroma. solitary bone cyst, osteoid osteoma, aneurysmal bone cyst, and giant cell tumor all rarely involve more than one bone and do not cross the joint surface. Multiple enchondromata can produce a very destructive process involving the bones of the hand, however, the joints are generally spared in contrast to a resorptive arthropathy. “’ The more malignant tumors such as chondrosarcomas and osteogenic sarcomas also do not commonly produce a destructive joint lesion. (3) Metastatic tumors. Metastasis to the small bones of the hands and feet is a very rare occurrence,‘7h.‘77 a statement supported by Dolich et al. in 1970, who could report only 35 cases in their review of the literature.“” The incidence of primary tumor involvement in their series was: lung and nasopharynx, 50”,,; genitourinary system, 20”“; breast, lo”,); parotid gland, IO”,;,; colon, 5”,,; uterus, 5°,1. Multiple bones were involved in five of 23 case reported by Kerin,“’ and in his series only one metastasis involved a joint. Karten and Bartfeld,lx” in 1962, reported a case of bronchogenic carcinoma with extensive metastasis to the hands, who at autopsy was found to have diffuse metastasis in the distal and middle phalanges of a finger with tumor infiltration into the synovial membrane.
230
SWEZEY
ET AL.
Fig. 31. Case 21. Surgery. Surgical correction of dorsal contracted toes mimics the changes of resorptive arthropathies. Resection of the metatarsal heads and base of the proximal phalanges in a patient with rheumatoid arthritis has resulted in a tapered appearance of the bone and widening of the joint spaces.
It is therefore evident that tumors very rarely involve the bones of the hands or feet and even more rarely involve the joints to produce a destructive lesion. No cases have been reported that could be confused with resorptive arthropathy. Case 21: (M.G.) (Fig. 31) Surgical resection of the metatarsal heads for rheumatoid arthritis resembling resorptive arthropathy. The patient is a 63-yr-old white female with a 3-yr history of rheumatoid arthritis which has involved most of the joints of her body with persistent activity. The most severely affected joints have been her hands, both wrists, the left elbow, the right metatarsals, and cervical spine. Positive physical findings are restricted to her joints. She has swelling and flexion contractures of the proximal interphalangeal and metacarpophalangeal joints, subluxation and hammertoe deformities bilaterally, and plantar calluses on both feet. Laboratory studies include: Hb. 13.8, WBC 7800 (normal differential), ESR 26, urinalysis, normal; RA latex test reactive, and serum protein electrophoresis normal. Aspirin has been her only drug therapy. In March 1971 she underwent bilateral resection of the metatarsal heads with excellent postoperative functional results.
PATHOGENESIS
In most destructive arthropathies, such as gout, leprosy, infectious and rheumatoid arthritis, the cause of the joint destruction seems obvious. Why then has the pathogenesis of the severe resorptive arthropathies escaped satisfactory explanation? In attempting to relate a reasonable pathogenesis for a clinical syndrome that encompasses multiple diseases, we have assessed concepts that have been suggested by previous authors and integrated their speculations with documented data in an effort to achieve an intelligent understanding and working hypothesis for the genesis of these uniquely destructive arthropathies. A number of components encompassing inflammation, vasculitis, neural
OPERA-GLASS
231
HAND
lesions, bone lesions, and mechanical out steroid therapy will be considered.
factors
including
joint
abuse with or with-
Lack of Inflammation Although pathological data on resorptive arthritis have been collected since the report of Marie and L&i, there is essentially no new light shed on the question of pathogenesis of these remarkable deformities.4~9~‘2,‘5~‘82’.23 Specimens from these patients showed fatty and fibrous tissue replacing the articular structures and sparing the neurovascular tissues.4*9,‘S.‘9.2’.23 Although pannus is a typical feature of rheumatoid arthritis, it has not been found in those joints demonstrating resorptive changes, and in patients with resorptive arthropathy pannus is usually seen in those joint areas not manifesting resorption and otherwise typical of rheumatoid arthritis. Although erosive osteoarthritis does not represent a classical disease in the group of severe resorptive arthropathies, the spectrum of pathological changes from acute inflammation to fatty and fibrous replacement of articular structures described in this disorder may well be representative of what one might see in several distinct noninfectious entities associated with severe articular resorption. Peter, Pearson, and Marmor, lo2 in their study of erosive osteoarthritis, noted a spectrum of pathology ranging from synociocyte hypertrophy, lymphocyte and plasma cell aggregation and fibrin deposition to cartilage and bone replacement with fibrous tissue. The pathology was variable and tended to reflect the cliniNone of their patients had sensory cal intensity of the inflammatory process. impairment to suggest a superimposed neuropathic condition. In two of their cases, thickening of blood vessel walls was noted. There is no documented evidence of the pathological progression of resorptive arthritis, but there is radiological documentation of a typical rheumatoid films, it appears process progressing to the resorptive state. ‘* From sequential that the pathological process begins as an otherwise typical inflammatory erosion (as indeed was seen in the erosive osteoarthritis patient of Peter, Pearson, and Marmor) and ultimately reaches a state of severe destruction replacing the former articular structure as well as the inflammatory lesion that destroyed it.lX.102
Vascular Lesion Of what significance are vascular lesions to this disorder? Paradoxically, ischemia and hyperemia have both been correlated with bone resorption while sluggish but highly oxygenated blood flow is accompanied by osteoblastic activity.‘83 I’46Increased regional blood flow (hyperemia) in active rheumatoid arthritis has been demonstrated. ‘*’ Pathological evidence of a unique or fulminant occlusive ischemic vascular lesion in resorptive arthritis is lacking. 4,‘s~‘9*2’Vascular occlusive disease of the digital arteries as well as sympathetic hypertonia in rheumatoid arthritis both are sufficiently common that if either the subendothelial digital arterial lesions of RA or sympathetic hypertonia were a major factor in the genesis of resorption one would expect a greater prevalence of the resorptive phenomenon.i88~‘92
232
SWEZEY
ET AL.
The possibility that inflammation in rheumatoid arthritis disturbs the glomus body which controls metaphyseal circulation and causes increased bone resorption and an “opera-glass finger” has been suggested.ls4 Other acral vascular reflex disturbances are associated with pathological joint changes such as pulmonary osteoarthropathy and local gigantism. In this connection, JohnsonLs4 mentions examples of massive osteolysis (neuropathic joints) of the hips developing in 6 wk without obvious preceding trauma in patients at bed rest. He argues that this must be on a neurovascular basis and that these cases tend to refute the commonly accepted concept that repeated minor trauma with disturbed healing is the chief mechanism leading to changes in the neuropathic joint. The possibility of a neural defect leading to inappropriate shunting of blood by a disturbed glomus mechanism in neuropathic joints is given additional credence by the recent observations of Kiss et a1.‘93 who have shown arteriographic evidence of arteriovenous shunting in Charcot joints. Drennan et al. removed the synovia (the presumed area of shunting) in an attempt to improve bone circulation and enhance the prospect for successful fusions of neuropathic joints.‘94 Angiographic studies of digital vessels in rheumatoid arthritis have revealed hyperemia in areas of synovial proliferations, occlusive changes and hyperemic changes in areas of bone destruction and erosion, but no clear evidence of changes in the venous phase of the examination which might suggest abnormal shunting, thus mitigating against a disturbed glomus mechanism as the basis of the resorptive process.‘88~‘90~‘9’ If, indeed, a disturbed osseous vascular supply due to altered glomus function plays a role in resorptive arthropathy, it must be presumed, for reasons discussed below, to be on the basis of a local glomus disturbance caused by the arthritic process rather than a more proximal neuropathic process. Neural Lesion The similarity of the severe destructive nature of the lesions in resorptive arthritis to the changes in the Charcot joints raises the question of not only a possible common vascular mechanism is these disorders, but a common neural one as well. Loss of proprioception and deep pain sensation are essential to the genesis of Charcot joints and this has not been observed in primary resorptive arthropathy. As mentioned earlier, in addition to resorption, the typical Charot joint is distinguished by a marked effusion, instability, cartilage and bone destruction and fragmentation, bone sclerosis and hypertrophic changes, and the presence of intrasynovial necrotic bone and cartilage fragments, while radiographically as well as pathologically, only destructive and resorptive L_23~85,92-195 These classical neuropathic changes are seen in resorptive arthropathy. joint changes are usually seen in weight-bearing areas at some time in the course of a Charcot joint, but sclerosis and hypertrophic changes are often absent in neuropathic arthropathy involving the small bones of the wrists and the hands.‘96 Marked effusion in association with digital resorptive arthropathy has not been observed. Further, in Hansen’s disease and in syringomyelia where sensation is lost, resorption in the hand typically, but not invariably, proceeds
OPERA-GLASS
HAND
233
from the distal digital tuft proximally, rather than from the proximal interphalangeal or metacarpophalangeal joints. 64 In the foot, however, where pressure effects are maximal on the metatarsal heads rather than on the toe pads, the destruction in Hansen’s disease is first seen in the metatarsophalangeal joints and the pattern of bone and joint destruction closely resembles that of exception to this pattern of initial digital resorptive arthropathy. 9o Another tuft resorption in Hansen’s disease is seen when lepromatous granulomata cause primary invasive bone destruction.9’ The lack of overt clinical evidence of gross neuropathy in all reported cases and the absence of microscopic evidence of neural involvement in resorptive arthropathy both mitigate a major role for a neurological deficit in this syndrome. l-z3 Impaired sensation and a reduction of motor nerve conduction velocity in 43% of 70 cases of rheumatoid arthritis was reported by Good et al.19’ Despite this data, Chamberlain and Bruchner were unable to correlate the degree of joint involvement with motor or sensory conduction impairment in patients with rheumatoid arthritis. I98 Although complete examinations are not available on all of our cases, where sensory, motor nerve conduction, and/or electromyography was performed on six of our patients, the only abnormalities found were some decrease in size of evoked potentials in two cases and polyphasic potentials in the opponens and abductor digiti quinti in one patient.“’ Further, the recognized protective effect of hemiparesis and anterior poliomyelitis on the progression of rheumatoid arthritis argues against the role of sensory and motor impairment aggravating rheumatoid arthritis, although admittedly in these instances there is a joint protective factor of disuse.2W,20’ One additional factor of likely significance was the successful healing of a surgical fusion of a thumb interphalangeal joint in our patient, V.R. The failure of bone fusion is a characteristic of the neuropathic joint.ly4 Destructive
Synovitis
Because of the initial intra-articular focus of resorption, it is logical to presume a primary role for the intra-articular inflammatory reaction in the genesis of joint resorption. The invasive destructive feature of the rheumatoid granulation tissue, resulting in articular erosions and juxta-articular cyst formation Rubenstein, and DixonZo2 studied the has been long appreciated.18’ Jayson, pressure in large intraosseous juxta-articular cysts in two patients with rheumatoid arthritis and observed that increased intra-articular pressure associated with muscle contraction was transmitted directly to these cysts, suggesting that these cysts were extensions of the intra-articular destruction. Further, in a patient with a large cyst or “geode, ” in the distal radius, relaxation of the clenched fist often was not associated with a drop in intrageodal pressure. This suggested that the communication of the cyst to the joints became plugged, thereby permitting prolonged high intracystal pressure to persist, adding pressure necrosis to pannus invasion in the destruction of juxta-articular bone. Pressure Efects That intact of rheumatoid
bone can be eroded in rheumatoid arthritis by extrinsic pressure nodules has been amply demonstrated by Dorfman, Norman,
SWEZEY
ET AL.
Fig. 32. Case 22. Rheumatoid arthritis. Subluxation at the proximal interphalangeal joints has resulted in severe deformity of the fingers and pressure erosions of the adjacent articulating bones, which mimics the bone loss from resorption. Typical pencil and cup deformities of the metacarpophalangeal joints are present. Incidentally noted is endosteal cortical thickening of the distal phalanges which is occasionally seen in rheumatoid arthritis.
and Smith.3’ The supracondylar scalloping of the osteoporotic femur by the patella, the notching of the ribs in some arthritics, and the erosions caused by contiguous subluxed bones as seen in our Case 22 (Fig. 32) are other apparent examples of bone erosion by extrinsic forces. 30,203,204 Intraarticular pressure has been shown to increase with muscle tension and weight-bearing stress to levels well above normal hydrostatic pressure. 202,204,206 In the severely eroded rheumatoid joints, the protective cartilaginous cover is lost. This leaves exposed the osteoporotic subchondral bone which has poor resistance to compressive forces and is thus highly vulnerable to erosive and destructive actions.207m209 Pain and Joint A buse Following Bywater’s suggestion, Castillo, Sallab, and Scott studied the relationship of osteoporosis to bone cyst formation and found an inverse correlation.2’0,2” They also noted that cyst formation in rheumatoid arthritis was more prevalent in males and attributed the severe destructive cystic changes to continued abuse of diseased joints by patients with a high pain threshold.2’2 These findings contrast with the female predominance and marked bone demineralization of resorptive arthropathy, although it is interesting that Colton and Darby8,‘8.20,2’2 comment on the lack of pain associated with giant granulomatous lesions of the femoral head and neck in rheumatoid arthritis, a phenomenon alluded to by Blum, Mather, and Smyth8~‘8~20 and which we have also noted in resorptive arthropathy. Corticosteroid Eflects Although it is clear that resorptive arthropathy was recognized long before the advent of corticosteroid therapy, it is significant that apparent abuse of diseased joints rendered less painful by repeated intraarticular steroid has been associated with a severe destructive arthropathy.‘261’29,‘3’
B
W
44 F
F
F
52
59
56F
72 F
24F
W.S.
E.G.
V.B.
V.R.
V.L.
M.H.
?
47
?
?
43
19
31
5
?
33
23
37
21
17
2U
16
19
41
37
16
15
19
20
11
YEAFLSIWRATION AGE OF AGE OF ONSET OF ONSET ARI'HRITIS RJ3SORPTION ARTHRITIS
* JuvenileRheumatoidArthritis * -losing Spondylitis
W
B
W
W
W
40 F
A.B.
B
27F
D.T.
F’A!l?IR?l’AGE SMRACE
TABLE III
+
+
ThUlUbS
0
+
+
0
0
0
0
+
0
Wrists Elbows
0
PhalanX
R-OX.
Distal ulna Cerv. Spine
ThUmbS
Left Elbow
0
5
SlTES OF PSOFUASIS *JRAONSEl' +IcA.S. RESORPTIVEARTHRITIS HAND FEEZCUl'HER Wrists + + Elbows Shoulders Wrists + + Shoulders Elbow Hip Neck Knee
RESORPTIVEARTHROPA!l'BYANDTHEOPERA-GUSSHANDSYNDROME
-
-
+
+
+
E.C.
V.B.
V.R.
V.L.
Sjogren’s
-
-
+
-
-
-
RA NOW
1:2560
+ -
+
+
l-l-l
+
-
+
+
+
+
+
1:80
-
+
1~2560
Mild
Mild
-
LATEX ANA L.E. PRESENCEOF PAIN PRE!P m ARJZAOF FZUTION TITRE RESO~IVEARTRROPATHY
I-
syndrome
I -I-
-
W.S.
M.H.
+
D.T.
PATIEwr-s.s.
Raynaud'sphenomenon,auto-amputation of right fifth toe, PSS.
OTBEX co-
Normal
Noxmal
Normal
Normal
Normal
Successfulsurgicalfusion I.P. joint right thumb.
Rheumaticfever at age 14.
Raynaud's30 yrs. Sclerodexma diagnosis 12 yrs. ago.
Clinical SI.Zin 1951. Past history of treated syphilis.TPI+.
Normal prior Mid-cervicalatraumaticspinal cord It.4Cord corn-Icompression.
Normal
slw3ORYExAM
TABLE III (cont.)
OPERA-GLASS
237
HAND
OSTEOPOROSIS
Juxta-articular bone demineralization is a classical feature of rheumatoid arthritis. Soila demonstrated that this occurred even in the absence of actual pannus invasion.‘24 Recent investigations have demonstrated calcium releasing factors in rheumatoid synovial culture media.*14 Generalized bone demineralization unrelated to steroids was observed in 27.8:,;I of 36 patients studied by McConkey, Fraser, and Bligh.2’5 Duncan et al.*“examined rib biopsies in five patients with rheumatoid arthritis who were taking salicylates, but who had not taken steroid medication. All of these patients showed greater bone resorption than bone production. Thus, at least in the rheumatoid, not only is the juxta-articular bone subject to demineralization, but a generalized oseoporosis and susceptibility to trauma may exist. Synthesis
Of’Pathogenesis
We believe it reasonable to hypothesize the following mechanisms in an attempt to explain the pathogenesis of resorptive arthropathy. Initially, an aggressive inflammatory synovial lesion progressively destroys the cartilage and underlying bone. In the destructive process, terminal neural sensory filaments may also be destroyed (thereby creating a partial articular anesthesia similar to the consequences of a synovectomy). Ultimately, the entire synovium is destroyed and when this stage is reached, the painful inflammatory reaction can no longer progress. The exposed demineralized juxta-articular bone is thus subjected to intraarticular pressure stresses in excess of that which can be tolerated without causing pressure necrosis and resorption. Bone healing and remodeling are insufficient to restore normal contours and the typical result is tapered (“pencil”) and reciprocally hollowed (“cupped”) bone ends.‘s4 The initial inflammatory reaction, once having destroyed the joint, subsides, leaving in its wake only fibrous and fatty remnants for discovery by the pathologist at his belated arrival upon this bleak scene. ACKNOWLEDGMENTS Permission to reproduce Fig. 23 was kindly given by the editorial Medicine. I would like to thank of the patients described
Mark Epstein, in this report.
M.D.
for his generous
staff of the Archives
assistance
in making
of Internal available
two
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H. H.: Essential J. Bone Joint
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in and Rheumatism
Arthritis
1972-1973
VOL. II, NO. 3
Resorptive
Arthropathy
the Opera-Glass Robert Stanley
L. Swezey. J. Alexander,
Hand David
and
Syndrome
M. Bjarnason,
and D. 6. Forrester
A
N INCREASING AWARENESS of the diversity of entities associated with a mutilating arthritis and the opera-glass hand (or the main en lorgnette syndrome) has led to a need for more precise categorization of resorptive arthropathies and their differentiation from other destructive skeletal disorders of the hands and feet. Since the first description of a case of resorptive arthropathy in 1888, there have been only scattered reports over the subsequent years. Although a variety of diseases have been associated with bone resorption, only a few demonstrate a resorptive process primary in the joint, characteristically in the hands and feet, and unassociated with clinically demonstrable neural or vascular defects. These cases in which the resorptive process not only caused a marked destruction of articular surfaces sufficient to cause a pleated appearance in the redundant loose overlying skin, but a remarkable tapering and “whittled” appearance of the shafts of the bone as well are considered to have primary resorptive arthropathy and include only certain of the collagen diseases and multicentric reticulohistiocytosis. To clarify this new classification, a review of the historical descriptions of resorptive arthritis in rheumatoid arthritis and in rheumatoid variants in association with the main en lorgnette syndrome, will be briefly summarized. A description of the clinical, pathological, and radiographic findings of the primary causes of resorptive arthritis and the main en lorgnette syndrome will be outlined, and contrasted with the findings in the other destructive arthritides and resorptive disorders of bone.
Supported
in part by a grant from the Arthritis
Foundation.
Robert L. Swezey, M.D.: Associate Professor of Medicine and Physical Medicine and Rehabilitation, University of Southern California School of Medicine. David M. Bjamason, M.D.: Fellow in Rheumatology, University of Southern Calijornia School of Medicine. Stanley J. Alexander, M.D.: Assistant Clinical Professor of Medicine, University of Southern California School of Medicine. D. B. Forrester, M.D.: Assistant Professor of Radiology, University of Southern Calijbrnia School of Medicine. 01973 by Grune & Stratton, Inc.
Seminars
in Arthritis
and Rheumatism,
Vol.
2. No. 3,
1972-l
973
191
192
SWEZEY
ET AL.
DEFINITION
Resorptive* arthropathy is defined as an articular disorder characterized by severe resorption of bone commencing at the articular surface or its margins and spreading proximally and distally. It usually predominantly affects the hands and feet and is typically associated with diffuse regional bony demineralization. It may have, in addition, infolded redundant overlying skin as a consequence of the loss of underlying bone.t (Fig. l-4).
Fig. 1. Case 1. Rheumatoid arthritis-main en lorgnette. skin overlying areas of bony resorption are evident.
Fig. 2.
Gross deformity
Case 7. Rheumatoid arthritis and systemic lupus erythematosis. areas of bony resorption is evident in all fingers.
and pleated
Redundant
redundant
and pleated
skin overlying
*Resorb: to lyse and assimilate (the substance of a differentiated structure previously produced by the body). Webster’s Third New International Dictionary of the English Language-Unabridged Springfield, Massachusetts. G. & C. Merriam Co., 1961. TOpera-glass hand, main or doights en lorgnette.
OPERA-GLASS
193
HAND
Fig. 3. Case 7. Rheumatoid arthritis and systemic lupus erythematosis. Severe resorption of the phalanges of the right fourth toe and associated pleated overlying redundant skin result in a doigt en lorgnette deformity in the foot.
Fig. 4. Case 8. Scleroderma (PSS) and rheumatoid arthritis. Classical main en lorgnette deformity. The skin overlying the digits is redundant but that over the wrist is somewhat thin, and areas of vitiligo can be noted. The skin has a somewhat shiny and unwrinkled appearance over the wrists and proximal dorsal hand area.
HISTORICAL
REVIEW
A review of the literature summarizes the case reports of resorptive arthropathy with sufficient juxta-articular bone resorption in most cases to cause redundancy and pleating of overlying skin (Table 1). The first recorded description of a case of resorptive arthropathy was by Bourdillon in his 1888 doctoral thesis entitled “Psoriasis et Arthropathies.“’ One of his cases showed resorption of several digits with almost complete disappearance of the right fifth toe. In 1903, Adrian reported a second case of psoriasis in a 44-yr-old white male who had severe destruction of the metatarsal and metacarpal phalangeal joints.* In 1906, Brumpt described a 35yr-old retarded Sudanese male with progres-
SWEZEY
ET AL.
: :
a!
3:
h
n2
R
P
7
OPERA-GLASS
HAND
195
196
SWEZEY
ET AL.
sive muscular atrophy and “doigts en lorgnette” or an “opera glass fingers” deformity which progressed with the muscular atrophy. This was the first use of the term “opera glass” to describe the infolded skin overlying resorbed bone. The exact etiology of this case, however, remains obscure.3 The contribution which attracted widespread attention to this curious entity was that of Marie and Leri in 1913 entitled “Une VariCtt de Rheumatisme Chronique: La Main en Lorgnette.” They described in detail the clinical, radiologic, and autopsy findings of a 70-yr-old woman with a 28-yr history of generalized arthritis which resulted in complete ankylosis of carpometacarpal joints, elbows, shoulders, knees, hips, and the vertebral column. Her digits were so resorbed that her hands and feet resembled “paws.” The skin over the digits was folded like an opera glass. Radiographs showed bony “transparency” and bony resorption of all bones of the hands, wrists, and of the distal radius and ulna. The pathology was characterized by fatty replacement of articular structures, some granulation tissue between bony structures, and sparing of tendons, nerves, and blood vessels except for mild endarterial thickening.4 This very complete description leaves only one lingering doubt, and that is whether or not this patient may have had ankylosing spondylitis in light of the mention of a complete ankylosis of the vertebral column in an admitted aged and infirm female. One of Zellner’s cases reported in 1928 was a man with severe psoriasis, typical resorptive arthropathy, generalized joint disease, and ankylosis of the cervical spine. Although the description of this patient is incomplete, as the sacroiliac joints and dorsal spine are not mentioned, it seems likely that this man had psoriasis, resorptive arthropathy, and ankylozing spondylitis. Under the title “La Main en Lorgnette,” Weigeldt,6 in 1929, reported a case similar to that of Marie and Ltri. He was the first to emphasize the tapering of the distal ulna. In 1931, Bauer and Vogl’ described a 64 yr-old male with psoriasis and resorptive arthropathy of the hands and feet. Their patient had typical clinical and radiographic features of ankylosing spondylitis. Blum,8 in 1933, used the title “Osteoarthropathia Mikroatrophicans” and described a 48-yr-old woman who had clinical evidence of subcutaneous nodules, normal serum calcium, phosphorous, uric acid, radiographic evidence of marked bone “atrophy,” and severe destruction of the distal clavicles and elbows, as well as of the metacarpophalangeal and proximal interphalangeal joints. The skin of the hands was loose and wrinkled and looked as though a “too large glove was being worn.” Stursberg,9 in 1935, reported two cases as “Polyarthritis Mutilans.” One had foot involvement which suggests the changes described later in the discussion of Werner’s syndrome. In 1936, Shlionsky and Blake” described an additional patient with psoriasis and resorptive arthritis involving the hands, wrists, and feet. Illustrations of their patient with the main en lorgnette syndrome received wide viewing as a result of having been published by Cornroe.” Nelson’s’* 31-yr-old female patient, reported in 1938, had intercarpal and interphalangeal fusion as well as resorption, cornea1 opacification, a renal calculus, normal serum calcium, phosphorous and phosphatase, and a parathyroid adenoma could not be determined with certainty. NO pathologic description of the joints was given.
OPERA-GLASS
197
HAND
In Schinz’s13 1939 text, a 45-yr-old
white female with “arthritis
mutilans”
in-
volving her hands and feet is illustrated. In 1941, Crain14 reported a 55-yr-old man with a 13-yr history of rheumatoid arthritis associated with marked joint resorption, no ankylosing, and death due to chronic renal disease of undetermined cause. No autopsy was obtained. Von Werthemann,” in 1945, described the pathological articular changes noted at autopsy of a 75yr-old patient consisting of fatty degeneration and minimal arterial involvement similar to that first noted by Marie and L&i. In 1946, Neilsen and Snorrason16 used the title “Arthritis Mutilans (Main et doight lorgnette)” and described the joints of six female rheumatoid arthritics with the syndrome. They noted that none of their patients had psoriasis nor evidence of neurological involvement and commented that in two of their patients there was a suggestion that mechanical factors may have played a secondary role in the joint destruction. in 1950, chose “Chronic Absorptive Arthritis or Solomon and Stecher,” Opera-Glass Hand: Report of Eight Cases” as their title. Two of their patients were psoriatics, one of whom lost the vision in an eye due to iritis. There were three male patients in their series. They introduce the term “cupping” to describe the hollowing out of the base of a bone at the joint surface opposite the tapered erosion of the distal head of the opposing articular surface. They also note, as had others dating back to the report of Marie and L&i, in both psoriatics and nonpsoriatics, the presence of fusion in joints showing severe resorption. They further remarked that joints not involved in the resorption process appeared to be otherwise typical of rheumatoid arthritis. In 1952, Smyth”’ reviewed the literature and reported four additional patients, including the first black (with the exception of the peculiar case of Brumpt). This second patient was noted to have a subcutaneous nodule (this one substantiated as a rheumatoid subcutaneous nodule by biopsy). Of particular additional interest is the autopsy report of a male patient who was described as having Still’s disease at age 23 (age of onset not given) and who died at age 33 of unstated causes. The articular findings at autopsy were reported as follows: “The cartilage and underlying cortical bone is replaced and held together by acellular fibrous tissue and fat. The remaining bony trabeculae were thin and the compact bone showed marked atrophy.” One of Smyth’s cases was also noted to have a unilateral protusio acetabuli (“Otto pelvis”). Serial radiographs over a 14-yr period demonstrated the radiographic manifestations of the pathological sequence of events in one patient. Joint narrowing and minimal erosions were present in a second metacarpophalangeal joint for 9 yr and then over the next 5 yr progressive resorptive cupping of the base of the proximal phalanx was observed. Silver and Steinbrocker” describe five patients, only two of whom had typical resorptive arthropathy of the hands and wrists; the other three demonstrated large cystic lesions located in the knee (distal right femur) in one, in the olecranon process of the ulna in another, and in the proximal head of the humerus in the third. Two of these patients had biopsies; one of the knee and the other of the shoulder, which showed the bone destruction resulted from a direct communication of granulation tissue from the eroded cartilage and bony surface into the bone. There was no evidence of a vascular lesion. Unfortunately, these
198
SWEZEY
ET AL.
biopsied cases were not truly representative of the usual acral form of resorptive arthropathy. “Arthritis mutilans” was the term used by MatherZo in June 1954 to describe a 36-yr-old female with rheumatoid arthritis who, over a two-yr span, developed pain-free hypermobile joints typical of resorptive arthropathy. In addition, she had Sjogren’s syndrome manifested by keratoconjunctivitis sicca, recurrent left parotitis, and a deficiency of tears. Biopsy of a subcutaneous nodule was reported as typical of rheumatoid arthritis. In 1955, Eisenstadt and Eggers” described a 17-yr-old male patient with recurrent heel and foot pain of four years’ duration. Attacks of foot pain were “unbearable” and ultimately a doight en lorgnette deformity of both fifth toes developed. Severe pain in the right knee occurred in the fourth year of his disease. Radiographs showed “cup and pencil” changes of the fifth metatarsophalangeal joint within 1 yr of onset. A year subsequent to this, further erosive changes were noted in the left heel and periosteal elevation of the shafts of the right second and third metatarsals was also observed. No mention is made of the possibility of psoriasis, ankylozing spondylitis, nor of Reiter’s syndrome. A biopsy of both fifth metatarsal joints revealed “yellow-colored cancellous bone partly replaced by a firm, shiny, greyish, soft tissue mass representing an osteolytic process with chronic inflammatory reaction and sclerosis of the blood vessels.” Friedman et a1.,22 in their study of the LE phenomenon in rheumatoid arthritis, described a 62-yr-old white male with psoriasis and several positive LE smears and a sheep cell titer of 1:28 who developed arthritis at age 45, which progressed to severe crippling and resorptive arthropathy by age 47. Their patient manifested ankylozing spondylitis.22 In 1966, Williams, Bland, and Lipson reported on a 74-yr-old woman with long-standing rheumatoid arthritis, a latex agglutination titer positive in a l:lO, 240 dilution, a biopsy-proven rheumatoid nodule, and marked resorptive arthropathy of the phalanges, carpals, proximal radii, distal ulnae, proximal humeri, and distal end of the right clavicle. In addition, and of particular interest, she had marked erosion of the odontoid, posterior displacement of the atlas (attributed to either loss of the anterior arch of the atlas or posterior dislocation of the atlas over the odontoid process with probable loss of its anterior arch), multiple cervical vertebrae subluxations, and a neurologic picture consistent with cervical spinal cord compression.23 Except for the nonspecific findings of an elevated sedimentation rate and a mild anemia, there has been little evidence to suggest a common pathophysiologic sequence of events in these cases of resorptive arthropathy. PRIMARY
Rheumatoid
RESORPTIVE
ARTHROPATHIES
Arthritis
Although resorptive arthropathy was first described in conjunction with psoriasis, it was Marie and Ltri’s description of the “main en lorgnette” syndrome in a patient with rheumatoid arthritis that initiated wide interest in the phenomenon.‘12
OPERA-GLASS
199
HAND
Prior to the widespread
use of the rheumatoid
factor
determinations
and the
ARA diagnostic criteria, there was no specific criterion that could establish a diagnosis of rheumatoid arthritis other than a biopsy-proven rheumatoid nodule.24.25 Only four of 37 patients described as rheumatoid arthritics (excluding the juvenile rheumatoid arthritics, the ankylozing spondylitics, and the cases with psoriasis) had clinical or biopsy-proven rheumatoid nodu1es.8~‘*~20,23 Rheumatoid factor in significant titre was reported in only one of the 37 cases. This patient had a biopsy-proven rheumatoid factor titer of 1:10,000.23 Since this is the only nonpsoriatic case reported since rheumatoid factors have been routinely measured and since the majority of patients with rheumatoid ‘nodules have significant rheumatoid titers, it seems likely that some of the 33 reported rheumatoid patients with resorptive arthropathy in whom no nodules are described represent examples of the usually benign seronegative rheumatoid disease which have developed the main en lorgnette deformity.25~28 Resorptive arthropathy occurs in patients with severe rheumatoid arthritis. The joints not involved in the resorptive process are typical on biopsy, X-ray, and clinical examination of rheumatoid arthritis.4,‘5,‘7,‘” The male/female ratio in rheumatoid arthritis is 0:3330.50: 1 and in resorptive arthritis is 0.55: 1.28(See Table 2.) The duration of disease to the onset of resorptive arthritis ranges from four to 50 yr with an average duration of 15 yr, and the average age of patients at the time of being reported is 46.5 yr. The distinctive clinical features of resorptive arthritis are evident bone resorption with loose and redundant overlying skin in the digits. Radiographitally, one observes dissolution of bone involving primarily the PIP and MCP or MTP joints. The DIP joints may be involved in the resorptive process in rheumatoid arthritis but are more often spared than in the psoriatic cases.‘8s29 The dissolution of bone progresses from the articular surface to create a tapering of the proximal articulating bone (licked candystick or whittled appearance) and a hollowing or cupping of the proximal articular surface of the distal bone. This may be associated with resorption of all or a part of the phalanges of one or more digits (doight en lorgnette).16 Frequently, the distal ulna, elbow, and occasionally the clavicle, shoulder, hip and cervical spine, including spinous processes, are involved.‘4.‘8*23 Compression or destruction of bone by a contiguous rheumatoid nodule has not been a factor in causing resorptive arthritis, although isolated reports of bone compression by this mechanism have been made.30,3’ Bone ankylosis is not an uncommon feature of rheumatoid arthritis occurring most notably in the carpal and tarsal bones, but occasionally in a digit as well. Resorptive changes may be seen in the same extremity manifesting ankylosed interphalangeal joints in both rheumatoids and psoriatics. Thus, new bone production and resorption are not mutually exclusive processes.8.‘8.32 The resorptive process tends to be insidious but, as mentioned, may progress rapidly and be well advanced in a 4-mo period.2’,32 The development of resorptive arthritis is commonly accompanied by a reduction in severity of pain and inflammation in the involved joints.8,20
200
SWEZEY
!lxBm
ET AL.
II
RESORPTIVE AFKt'HROPATHy AND THE OPEFhGLASS HAND SYNDROME
NO.OF CASES DURATION OF ARTHRITIS IN YEARS*
YEARS OF ARTHRITIS Average Range x5.3
4u57
MEAN AGE OF ALL PATIENTS INYEARS
49/57
46
WY++
4S
lS/lS”
45
IBAN
AGE OF FEWLES +
EAN
AGE OF MALES +
WITE
s/50*
RACE JINFh?LlE FJ-IEU?Wl?OlDS INKYLOSING
SPONTZLITIS
34
4 moe.+O yrs.
WQ?GE 21-75
BUCK
2
4 4-
SJOGREN'S SYND3.0~
1
PSORIASIS
16
*
Duration does not necessarily imply duration of arthritis prior to onset of Resorptive Artbropathy.
++
Number of cases in which information is available out of total number of cases reported.
m
Eleven additional cases had either sacroiliitisor ankylosis of vertebrae, but insufficientdata y,yas given to accurately categorize them tither.
+
Male/female ratio = 0.55/l.O
OPERA-GLASS
HAND
201
Because of extensive destruction in the resorptive arthritic process, many patients have become severely crippled; but a common observation is a surprising degree of useful and dextrous function in spite of incredibly deformed hands and other joints, which is probably attributable to the subsidence of articular pain in the destroyed joints.8,2” (‘USCI: (A.B.).(Figs. 5-8) Classical rheumatoid arthritis with high titer rheumatoid Factor and Sjogren’s syndrome. The patient is a 40.yr-old white female who had the onset of monoarticular rheumatoid arthritis involving the right elbow in 1950 at age 20. Over the ensuing 3 mo the disease became generalized, resulting in a nonambulatory status, and she developed a progressive deforming mutilating arthritis of all extremities, particularly the fingers. In 1953, she had surgery on both knees for correction of flexion contractures and postoperatively was able to ambulate with crutches until 1963, at which time she again became confined to a wheelchair. In 1967 she first noticed burning and dryness of her eyes and in May 1969 she was noted to have classical changes of Sjogren’s syndrome on slit lamp examination. In July 1968 she developed cervical cord compression secondary to cervical spine involvement with rheumatoid arthritis whtch was successfully treated conservatively with a cervical collar. Musculoskeletal examination at that time showed generally greater than 50”,, loss in range of motion in all joints, with Rexion contractions of all extremities, ulnar deviation of the fingers, and main en lorgnette deformity of both hands. Neck motion was restricted to 15” of lateral flexion; 35” forward flexion: 15” extension: and 60” of rotation. There was generalized decrease in motor strength. normal deep tendon reflexes, flexor plantar responses, decreased sensation to touch and pain over both arms. and equivocal findings of mild spasticity in the legs. Cervical spine X rays showed destruction of the third and fourth cervical bodies with destruction of the interspace between C2 and C3 and between C4 and C5 and extensive destruction of the posterior spine with slight anterior displacement of the bodies of C2. 3. and 4. Physical examination was otherwise within normal limits. Significant laboratory studies were Hb. 11.7, WBC 9000 (normal differential), ESR 28, albumin 2.5, globulin 3.6. antinuclear antibodies negative, serum complement normal. rheumatoid factor positive in 1:163,840 dilution LE cell preparation negative. Other significant illnesses include a vagotomy and pyloroplasty in April 1967 for a gastrointestinal hemorrhage, a gastroJejeunostomy in March 1969 for a recurrent hemorrhage, and an acute paranoid psychosis in November 1968. Treatment of her rheumatoid arthritis was initially steroids for the first 2 yr and primarily aspirin since that time
Fig. 5. Case 1. Rheumatoid arthritis. Concentric resorption of the phalanges of the second finger has caused foreshortening and overlap of the soft tissues resulting in the classic “opera glass” deformity. Resorption of the second metacarpal head and subluxation of the base of the phalanx add to the deformity. The third and fourth fingers demonstrate an additional cause of destruction. Subluxation of the middle phalanges has caused saucerlike pressure erosions as their abnormally positioned ends erode the proximal phalanges. The wrist demonstrates subluxation as well as resorptive changes of the distal radius and ulna.
SWEZEY ET AL.
Fig. 6. Case 1. Rheumatoid arthritis. Progressively more severe destruction of each digit demonstrates the series of changes that occur in the formation of a pencil and cup deformity. Although the first, second, and third metatarsophalangeal joints appear ankylozed. oblique views verified this to be destruction of cartilage with secondary irregularities of the articular ends of the bones. Resorption of the heads of the fourth and fifth metatarsals has caused widening of the joint spaces. lnvagination of the pencilled metatarsal bones into the base of the fourth and fifth proximal phalanges ultimately produces the pencil in cup deformity.
Fig. 7. Case 1. Rheumatoid arthritis. The mutilating effects of rheumatoid arthritis are not confined to the hands and feet of this patient. Tapering of the radius and ulna and remodeling of the distal humerus wi?h total destruction of the joint are apparent.
203
HAND
OPERA-GLASS
Fig. 8. Case 1. Rheumatoid arthritis. Resorption of the femoral head and protrusion of the neck into the pelvis may reflect the tendency toward generalized resorption of bone. A superimposed avascular necrosis cannot be excluded without a series of films to document the progression of pathological events.
(V.B.)
(‘use 2:
(Figs. 9 and 10) Classic rheumatoid
arthritis
with resorptive
anthropathy
mal in the hands, but remarkable
in the cervical spine. The patient
the onset of rheumatoid
in 1954 at age 43. The disease began in her shoulders,
arthritis
wrists, and hands and progressed to generalized present, she has had persistent she developed
subcutaneous
Sjogren’s syndrome
moderately
nodules
active
around
since 1963. Treatment
both
Of significance
I4 consisting of arthritis, examination
multiple ecchymotic examination sternal border
skeletal examination
radiation
heart
palmar grade
creattnine
I. I, gamma-globulin
0.6,
with
erythema;
albumin
2.6,
component,
severe rheumatoid
motion,
of both knees, swelling
factor I /2560, normal, with
anti-DNP
ulnar
resorptive
left
deviation
interphalangeal
joints,
motion
ol
surgical scars.
alpha*-globulin antibody
the
Musculo-
IO” Rexion contracture
bilateral
platelets
chest X ray normal. early
negative.
On with
HEENT
along
of the eyes showed
differential), 0.5.
140/80.
and decreased
joints with overlying
6900 (normal
arthritis
abdomen
metatarsal
symptoms.
heard
joints and proximal
alpha’-globulin
74 mg Y;, ECG
murmur
ol
fever at age
appearance
blood pressure
systolic
Slit lamp examination
12.3, WBC
2.0, rheumatoid
tion positive, cryoglobulin compatible
was normal.
tests: Hb.
symptoms
5 yr of her disease
cushinoid
of the neck and shoulders;
of the metacarpophalangeal
examination
normal.
a moderately
II/VI
and with no diastolic
of ths hips, synovial thickening
Laboratory
in her disease,
She has never had any neurological
both ankles, and swelling of the metatarsophalangeal Neurological
for the initial
swelling of both wrists with decreased
of the fingers, subluxation
keratitts.
aspirin
presented
revealed decreased motion
of both elbows, synovial normal motion
and chorea.
1969, the patient
chest negative,
without
Early
elbows,
Up to the
5-10 mg day). In 1964 she had bilateral
skin lesions and bilateral
negative,
relapse.
and she has manifested
in her past history is an episode of acute rheumatic
carditis,
in November
elbows
mini-
female who had
over a short period of time.
disease without
was mainly
and then steroids for the past IO yr (Prednisone head resections.
involvement
is a 59-yr-old
tilamentary
normal, 0.8.
urinalysis
beta-globulin
negative,
LE cell prepara-
X-rays
of the hands were
changes.
The
cervical
spine
204
SWEZEY
ET AL.
Fig. 9. Case 2. Rheumatoid arthritis. Generalized cartilage destruction of the metacarpophalangeal joints and erosive changes of the proximal and distal interphelangeal joints have resulted in severe malalignment, and a wavy contour of the fingers mimicking an “opera glass” hand. Resorptive changes of the proximal phalanges of the thumbs and the distal ulna are also present.
revealed marked resorption of the spinous processes of CS. In November 1965 the patient developed a large ulcer on her lower right leg after a cat scratch, which slowly healed after conservative treatment. Case 3: (V.L.) (Fig. 1 I) Seronegative rheumatoid arthritis, Sjogren’s syndrome, and resorptive arthropathy. The patient is a 72-yr-old black female who had the onset of rheumatoid arthritis in 1930 at age 31. The disease began in her thumbs and progressed to generalized joint involvement over the next few years. By the time she was first seen in our clinic in 1966, she had a supracondylar osteotomy of the right knee. She has had Sjogren’s syndrome since 1967. In June 1969 she had an episode of iritis. Over the past 4 yr, she has complained of 223 hr of morning stiffness with intermittent paints and occasional swelling in various joints. She has never had a subcutaneous nodule. She has had no neurologic symptoms. Medication over the years has been mainly aspirin and she has never had gold treatment. Examination in August 1966 was as follows. Examination of the head, chest, heart, and abdomen was negative. Musculoskeletal examination showed moderately decreased range of motion in the neck, shoulders, and elbows. The wrists were subluxed; the fingers showed ulnar deviation, subluxation of the metacarpophalangeal joints, and telescoping of the phalanges. The hips, knees, and ankles all showed decreased range of motion.
Fig. 10. Case 2. Rheumatoid arthritis. The lateral cervical spine demonstrates erosion of the odontoid and subluxation of the atlantoaxial joint. Rarely, erosions of the spinous processes occur as well; an example is seen at C5.
OPERA-GLASS
HAND
Fig. 11. Case 3. (V.L.) Rheumatoid arthritis. Generalized osteoporosis and severe interphalangeal joint destruction are present. The distal interphalangeal joints are narrowed and small erosions are present. Concentric resorption of the distal ulna and a pressure erosion of the proximal phalanx of the thumb add to the deformity.
No subcutaneous
nodules were present and neurologic
of the hands was normal, Bengal staining laboratory
11.9, WBC
4.3,
urinalysis
x ray was normal
and ECG
DNP
antibody
Case 4:
normal,
negative.
arthritis (V.R.)
keratitis.
changes.
antibody
rheumatoid
arthropathy.
rheumatoid
in 1935 at age 19. shortly
1968 she noticed syndrome mainly
marked
for many
of aspirin:
arthroplasty
The patient
rapidly
progressed
she has never had
in September,
fingers. subluxation phalangeal
joints,
after
50. albumin
normal.
Chest
was negative,
serum complement
anti-
normal.
and
changes compatible
with
termination
syndrome,
Her
hands
She has a history
treatment.
in
disease
and
since the onset. and from nodules.
re-
fusion
who had the onset of
of a pregnancy.
srlastic implants
interphalangeal
and focal
thumb
her feet. knees, and
of her hands. steroid
wrists,
Sjogren’s
then
1963 to
of Sjogren’s
Treatment
has consisted
She has had
a metatarsal
of the metacarpophalangeal
jornts of the right long and ring tingers,
fuston.
1968, showed: Musculoskeletal
contracture,
wrests had a total
of the metacarpophalangeal dislocation
factor
representative
ESR
enrymes
white female
to be active
parenteral
joints of both hands and of the proximal Examination
serum
destructive
She has never had subcutaneous
and a right thumb interphalangeal
are
normal,
successful interphalangeal
to involve
increase in the deformity
of the left foot, fusion of both
bows had a 20” flexion
negative,
is a 56-yr-old
disease has continued
years.
following
Rheumatoid
arthritis,
an area of resorptive
The
sensory examinatron
changes in the hands.
in the hands, with a subsequent
became generalized.
The 2.5,
showed
sorptive arthropathy arthritis
including
2 mm of wetness and the Rose
platelets
phosphatase
X rays of the joints
12) Seronegative
began in the left shoulder,
less than
differential),
alkaline
anti-DNA
with resorptive (Fig.
pun&ate
(normal
showed nonspecilic
positive undiluted,
LE cell preparation rheumatoid
4800
examination,
test showed
of the eyes demonstrated
tests: Hb.
3.8, globulin
The Schirmer’s
joints.
of the metacarpophalangeal
system: shoulders
of 20” motion,
flexion contractures and proximal
ulnar
abduct
to 80”. el-
deviation
of
of the proximal interphalangeal
all
inter-
joint
of
SWEZEY ET AL
Fig. 12. Case 4. Rheumatoid arthritis. Although subluxation of the metacarpophalangeal joints and the wrist are the most prominent abnormalities, there is resorption of the proximal phalanx of the thumb and subluxation of the distal articulating bone.
the right thumb and NalebufT 2 deformity of the left thumb, Rexion contractures of both hips and knees, No subcutaneous nodules were present. She had marked erythema about her fingernails and palmar surfaces. The rest of the examination including the neurological was normal. Schirmer test showed 0 and 2 mm of wetness. Laboratory tests: Hb. 13.0, WBC 8200 (normal differential), platelets normal, urinalysis normal, ESR 47, creatinine 0.9, albumin 3.5, globulin 4.5, rheumatoid factor negative, anti-DNP antibody positive in I:4 dilution, anti-DNA antibody negative, serum complement normal, LE cell preparation positive. Chest X ray showed linear densities at both changes with resorptive bases, probably old. X rays of her hands showed chronic destructive changes in the interphalangeal joints of the right thumb. In January 1970 she had an acute myocardial infarct with no previous history of heart disease. In December 1971, surgical bone fusion of the right interphalangeal joint of the thumb was successfully performed and this represents the only report to our knowledge of a surgical fusion in a patient with resorptive arthropathy.
Psoriatic
Arthritis
The propensity for psoriatic arthritis to lead to bone resorption has been rePsoriasis and typical rheumatoid arthritis coexist in peatedly observed. 1~2~7~10~‘7~32 psoriatic arthritis about 18% of psoriatic patients.33,34 Features distinguishing from rheumatoid arthritis are: the usual lack of rheumatoid factor and rheumatoid nodules; the greater prevalence of distal interphalangeal involvement; the greater tendency to asymmetry of joint involvement; and the increased tendency to bone proliferation in the psoriatics.10,32-36 Psoriatic skin lesions occur prior to or simultaneous with the onset of arthritis in the vast majority of patients. 32 Nail changes have been observed in 80”/,
OPERA-GLASS
HAND
207
Fig. 13. Case 5. Psoriatic arthritis. Both rheumatoid and psoriatic arthritis may cause severe resorptive changes in the hands. Several radiographic distinctions between the two diseases are illustrated. (1) Bony mineralization tends to be maintained. (21 Bony ankylosis of the interphalangeal joints is far more frequently seen in psoriatic arthritis. (31 Extensive involvement of the distal interphalangeal joints is present. (4) Widening of the joint space from excessive fibrous deposition is present at the fourth and fifth proximal interphalangeal joints.
of psoriatics who developed arthritis. 32 Psoriatic erythroderma has been reported to be more common in those patients developing psoriatic arthritis and was reported to occur in four of seven cases of resorptive arthropathy in one series.32 Peripheral joint ankylosis has been noted in almost half of the psoriatics developing resorptive arthropathy. 32 Sacroileitis has been noted in about i of patients with Reiter’s syndrome and the close relationship of these syndromes
Fig. 14. Case 5. Psoriatic arthritis. Aesorptive changes in the foot are most extensive at the pressure points and thus deform the metatarsophalangeal joints. Bony ankylosis of the proximal interphalengeel joints and severe erosion and widening of the distal interphalangeal joints distinguishes this pattern of destruction from the changes of rheumatoid arthritis. Deposition of fibrous tissue at the interphalangeal joint of the great toe with resultant widening of the joint space is a frequent finding in psoriatic arthritis.
208
SWEZEY
ET AL.
has been documented.34.37*38 Two of Green’s39 patients with psoriasis and severe erosive arthritis gave a history consistent with Reiter’s syndrome, and one patient with Reiter’s syndrome along with resorptive arthropathy has been reported .40 Case 5: (L.B.) (Figs. 13 and 14) Psoriasis with arthritis and resorption of the distal phalanges. The patient is a 49.yr-old white female who has a 33-yr history of psoriasis for which she has been treated with local steroid creams and parenteral steroids. She also has a 30-yr history of associated arthritis with involvement of the hands, feet, and neck with resultant prominent deformity in the hands and feet. Significant physical findings demonstrated psoriatic plaques over her back, buttocks, trunk. and extremities, pitting and grooving of the nails, and deformity of the hands with resorption of the distal phalanges (right greater than left) and shortening of the toes. She has no other joint involvement.
Ankylozing
Spondylitis
It is interesting to note that although sacroiliitis and resorptive arthropathy have been observed to coexist commonly in psoriatics and juvenile rheumatoid arthritics, evidence for coexistent ankylozing spondylitis or entereopathy with sacroiliitis and resorptive arthropathy is found in only seven cases.32~34~41~42 Zellner’s 5%yr-old male patient with severe psoriasis, generalized disease, and ankylosing of the cervical spine is presumably a case of ankylosing spondylitis.’ One of Soloman and Stecher’s patients, a 33-yr-old woman with juvenile rheumatoid arthritis, was described as having an ankylosed spine.” Two additional patients with ankylozing spondylitis, psoriasis, and “arthritis mutilans” are cited by Wilkinson and Bywaters, 43 but the only details mentioned are fusion of the metatarsal phalangeal and interphalangeal joints. The patient described by Friedman et al. was a 62-yr-old white male who had psoriasis, positive LE smears, and ankylosing spondylitis. ** Smyth described a 23-yr-old man with Still’s disease, resorptive arthropathy, and ankylosis of the spine, elbow, wrists, knees, and ankles.‘* A black female with ankylosing spondylitis and marked cystic lesions confined only to the humerus but no peripheral bony resorption, has been reported. I9 Marie and L&i’s patient was an elderly female and although no mention of spine radiographs is made, they describe her as having a complete ankylosis of the vertebral column. However, because of the relative rarity of ankylosing spondylitis in females, and the advanced age (70 yr) of their patient, it seems reasonable to postulate that the clinical appearance of ankylosis of her spine may have been attributable to osteoarthritis and contractures secondary to prolonged invalidism. Juvenile Rheumatoid
(JRA )
Resorptive arthropathy has been observed in three cases of juvenile rheumatoid arthritis.‘2~‘6~i7 The onset of arthritis in one case began at the age of five and the onset of the arthritis for the other two in the second decade.16 Aside from the resorptive arthropathy, no other unique features of the patients with juvenile rheumatoid arthritis have been noted.‘2~‘6~‘7 Case 6; (M.H.) (Fig. 15) Juvenile rheumatoid arthritis with resorptive arthropathy. The patient is a 24-yr-old female who had the onset of rheumatoid arthritis in 1952 at age five with pain and swelling in the ankles. This soon progressed to generalized joint involvement requiring steroid therapy during the first year of her disease: however, she developed severe cushinoid side effects and the steroids had to be discontinued. Over the next few years she developed progressive deformity of her wrists, hands. fingers, and elbows. At age six she had poliomyelitis which
OPERA-GLASS
HAND
Fig. 15. Case 6. Juvenile rheumatoid arthritis. Occasionally, severe destructive changes and erosions occur in juvenile rheumatoid arthritis and resorption of bone is extensive enough that overlapping of soft tissues causes an “opera glass” hand. Subluxation of the metacarpophalangeal joints and bony ankylosis of the wrist are additional stigmata of the juvenile onset of an inflammatory arthritis.
lel’t her
with residual
weakness in the left knee. At age 13 she had a supracondylar
the left knee and was treated with In
1966 she had a left Achilles
arthrodests.
The general activity
with chronic deformities fever. skin
rash.
nor
cutaneous nodules. microenathia.
without other
swelling
both
ESR
56,
is otherwise
platelets
of juvenile
rheumatoid
hands. Functionally.
ankle\
normal.
negative. 1.E cell preparation istic
thumbs
with
associated
at present she can stand for about
as an active college student
examtnation
13.3. WBC normal.
resorptive
I
9600
tests
although
in
both
bilaterally
contracture,
Joint
joints
is normal.
(normal
normal,
the arc
General
differential).
rheumatotd
factors
X rays were character-
changes rn the joints
of the
hr. can walk with crutches for one block,
She has moderate stiffness
write.
Joints
knee has a flexion
functton
negative, chest X ray and ECG arthritts
of both elbows,
and the metatarsophalangeal Hh
liver
suh-
1961 sholced.
appearance and havmg the feeling 01
the right
studies:
normal,
pain and is able to feed herself.
tain herself
triple
decreased motion
contractures
and metacarpophalangeal
have no motion
hut uses a wheelchair for most of her activities. has little
ankle
has never had in July
decreased mobility,
with a telescoping
Laboratory
urinalysis
She
Examination
nodules arc present and the neurological
normal,
of
of the knee.
her disease she did not have
disease.
asptrin.
with markedly
both hips have decreased motion.
swollen. No subcutaneous examination
of Still’s
been with
no motion,
the fingers are foreshortened ankylosed.
fracture
sttffness
and also a bilateral
or much pain. Throughout
and abduction to 45”. 20” flexion
with flexion
being very fratl.
procedure
characteristic
has mainly
wrests held in 25” of Rexion uith
left knee is
tn a residual
of her disease appeared to decrease in 1967, and she was left
lindings
Treatment
resulted
tendon lengthening
neck held in 30” Hexion
both shoulders suhluxed.
spica casts. This
and assist
in transfers.
recently
she is having
in the mornings She
hut
is able to main-
increasing
difficulty
in
moving her upper extremities.
Other
Collagen
S~~stenlic
Diseases
Lupu.~
Er~thrn~atosis
is characterLed
by a paucity
ceptions
are
to this
recognized
(SLE):
Systemic
of destructive and
so-called
articular
lupus
erlthematosis
involvement.‘“~”
crossover
cases
with
(S1.k.) hlanl
rheumatoid
ex-
210
SWEZEY
ET AL.
arthritis and other “collagen” diseases are well documented.46m4y Positive LE smears and antinuclear antibodies occur commonly in patients with rheumatoid arthritis, but these patients have a course typical of rheumatoid arthritis.50%56 It would, therefore, not be unreasonable to assume that a coexistent case of SLE and resorptive arthropathy would occur. None have so far been reported; however, we have observed one patient (W.S.) who demonstrates this combination in addition to Sjogren’s syndrome. Cuse 7: (W.S.) (Fig. 16) Systemic lupus erythematosis and rheumatoid arthritis with resorptive arthropathy. The patient is a 44-yr-old black female whose illness began in April 1951 with migratory arthralgia and a butterfly erythematous rash on her face. That same year she developed pieurnis and was diagnosed as having SLE. In June 1952 she presented with pleuropericarditis, butterfly rash, alopecia in the temporal region, and anemia. SLE was again diagnosed although no LE ceils were seen on bone marrow or peripheral blood smear. Laboratory studies of record showed: Hct 30, WBC 5,000. urinalysis normal. She was treated with aspirin. In 1952 she also developed pain and swelling in the joints of her hands and feet which progressed to severe deformtty over the next 10 yr. InHammatory involvement of the elbows, knees and ankles had been present but dtd not result in deformity. In 1956 she began complaining of dryness of the eyes and mouth persistmg to the present. Eye examination in April 1968 showed the presence of healed and vascularized cornea1 ulcers. Schirmer’s test in June 1970 showed wetness to 35 and 22 mm. There is no history of Raynaud’s phenomenon, subcutaneous nodules, dysphagia, scleroderma, or other skm changes. When first seen at the LAC/USC Medical Center in 1968, examination was unremarkable except for severe shortening of all the digits of her hands and the fourth and fifth toes of the right foot. The skin over the digits was loose and redundant. The involved digits demonstrated the classical telescoping appearance of the main en lorgnette deformity. All involved joints were painless. Examination of other joints was normal. Significant laboratory studies showed: Hb. 10.9, WBC 4,000, ESR 54, BUN6, normal urinalysis, SPEP normal except for a gamma globulin of 3.1, LE preparation positive, anti-DNA-protein antibodies I: 16, anti-DNA antibodies negattve, rheumatoid factor 1:80, serum complement 640 (normal). Chest X rays and pulmonary function tests were compatible with restrictive lung disease secondary to tibrosn. Esophageal X ray showed a dilated atonic esophagus. Renal function has always been wtthin normal hmits. Treatment since 1968 has been only aspirin. Significant past history includes a htstory of syphihs diagnosed and treated in 1951 with 43 million U pencillin. In 1970
I
Fig. 16. Case 7. Systemic lupus erythematosis and rheumatoid arthritis. Cartilage loss and resorptive changes are limited to the fingers and have caused severe loss of bone and redundancy of the soft tissues and the classic picture of an
OPERA-GLASS
the VDRL
211
HAND
and TPI were positive.
It was felt that
the pattent
had
received
adequate
therapy
for
her syphilis.
Progressive Systemic Sclerosis (PSSI: Like SLE, progressive systemic sclerosis (PSS or scleroderma) has been identified with a lack of invasive synofeature of subvitis or pannus. jjs9 The unique, but by no means constant of positive rheumatoid synovial fibrosis is peculiar to PSS.j7.‘” The association factors and a more aggressive synovitis has been reported.“,j’ Positive antinuclear antibodies and a close clinical relationship to SLE are also frequently Seen ,48.49X60,61
Distal digital resorption is a well-recognized feature of PSS.62,67 This has been attributed to ischemia associated with Raynaud’s phenomenon and the attritional effects of the sclerodermatous process in the skin overlying the phalanges.62,68 An apparently self-limiting form of Raynaud’s phenomenon and digital tuft resorption has been reported in men exposed to polyvinyl chloride.h9 An intriguing experiment of nature has been reported in which a patient had a traumatic amputation and successful reattachment of the distal half of her left fourth finger at age three and subsequently developed scleroderma at age 50. Raynaud’s phenomenon and resorption of the fingertips and distal phalanx occurred in all but the formerly amputated finger.” Although some sparing of a neurovascular defect by the amputation and reanastomosis is clear, the exact mechanism remains obscure. PSS, indistinguishable from rheumatoid arthritis. has been described in only two patients, and the evidence presented for scleroderma in one of these cases is incomplete. “Her fingers were cold, clammy. and cyanotic, suggesting sclerodactylia and Raynaud’s disease, for which bilateral sympathectomies were performed.“” The second case reported by Dubois et al.49 is again described in this report (Case 8) and probably represents an example of the coexistence of rheumatoid arthritis and PSS (see Fig. 16). Case 8: (D.T.) (Fig. 17) Progressive systemic sclerosis and rheumatoid arthritis with resorptive arthropathy. This patient is a 27-yr-old black female whose illness began at age I6 -81th prominent swelling and tenderness of the small joints of the hands, knees. and ankles. She was initially diagnosed as having rheumatoid arthritis because of an associated positive latex fixation test; however, over the next year she developed the typical skin changes of scleroderma Involving her face and hands. She also experienced intermittent Raynaud’s phenomenon. Skin biopsy at that time was read as compatible with scleroderma and an upper gastrointestinal X ray showed lack of esophageal peristalsis. Over the next 3-4 yr her arthritis progressed, generalized. and resulted in deformities and flexion contractures so that she was confined to a wheelchair. A major problem from the second year of her illness to the present has been recurrent skin ulcerations over the knees, lateral and medial ankles. soles of the feet, and tingertips. In October 1967 she suffered an autoamputation of her right fifth toe. In 1968 she complained of dryness of her eyes and a Schirmer’s test showed 9 mm of wetness in one eye and I mm in the other after 5 min. Examination of the cornea was normal. Musculoskeletal examination in October 1968 showed micrognathia. limitation of neck rotation, main en lorgnette changes in the hands, moderate flexion contractures in wrists. elbows, shoulders. knees, and ankles. Neurologic examination was normal, including sensory exammation of the hands Phystcal examination was otherwise normal. Significant laboratory studies tn August 1970 were’ Hb. 12.4. WBC 7900, ESR 47, normal urinalysis, BUN 10, normal serum enzymes. SPEP normal except for gamma globulin of 2.0. Representative LE preparations have been positive. anti-DNP antihodies positive undiluted, anti-DNA antibodies negative, and rheumatoid factor was 1:2,560 She has had no pulmonary disease clinically or on X ray and no leukopenia suggestive of a Felty’s syndrome Treatment over the years has consisted mainly of steroids (Prednisone up to 20 mgiday) and she is presently taking 5 mg/day.
SWEZEY
Fig.
17.
Case
progressive gressive
systemic
toid arthritis syndrome have
in
Fig. 18.
Case
are sule
present.
the frequency of the may
in which
proximal
be
calcium
occurrence
in the related deposit.
hand.
tissue
within when
the
the
of
sclerowasting
joint
cap-
compared
subcutaneous Erosion
phalanx to
systemic
and soft
Calcification
is a rare
are seen
9. Progressive
osteoporosis
collections
of the the
contiguous
with
distal
end
second
finger
soft
tissue
sclerosis
this
bone
as
as
well
well
as
as
of
and
of prorheuma-
to Sjogren’s
Resorptive
loss
ulna.
arthritis
Elements
in addition
patient.
marked
ance to the distal
sis. Diffuse
Rheumatoid sclerosis.
are present
caused
phalangeal
8.
systemic
ET AL.
changes
metacarpal
a whittled
and
appear-
OPERA-GLASS
(‘are
Y.-
(L.C.)
to represent white
HAND
(Fig.
18) Classic
an example
female
developed
213
who
had
arthritis
the onset
involving In
Increasing
sktn changes
had intermittent
skin
of
or pulmonary
ulcerations
Increasing involvement.
main
She has noticed
been mainly continued
aspirin because
Involvement ulcer\
and
pain
pain
although
of gastrointestinal
on her
side
abdominal.
lingertips
and
and neurological
metacarpophalangeal. digital right
tips.
mal.
Thcrc
elbow.
motion
Hand
X rays showed
In the left
proximal
of the proximal bowel
anti-DNP
X ra!
tionb of the left angle but normal
and
dilation
folate
positive
in
around
There
carotene
.joints.
Esophagus
X rays showed
colon.
vasculature.
Chest
bowel:
knees.
X ray ahoued
of
her
and
time
slight
nor-
50 300).
75”,,. rheumutold
deatructlw v,ith
changes ahoued
ol the
of the decreawd
platelets
antibody
changes
enema
se\cral
contracture
dill’erential).
classical
blunting
on
of her wrist\.
72 mcp IA0 (normal
mutllans
barium
h,l\
bc dis-
of hcalcd
present
resorption
and PIP joints,
arthritis
\mall
zre
antl-DNA
foreshortening.
IS the
sclerodermatou\ tvidence
flexlon
5 mg “,,. prothrombin ,yj dilution.
ankles.
do not clear on coughing.
90’
4600 (normal
feet,
had to
is ankylosts
and
jhe
Treatment
typical
marked
joints.
the Nrlsta
Four CYS.
deformit)
but
and hand\.
with
with
of the proximal
transverse
pulmonary
was trlcd
of the hip\ serum
knees,
months.
feet. Telangiectasia
joints
I I .5. WBC
14. K 4.2.
soft tissue calcification phalanges
few
shows
are negative.
motion
315), serum
antibody
interphalangeal showed
normal BUN
I35
Penicillamine
of her
01
She ha\
cardiovascular.
are the
the past
of her shoulder
teats: Hb.
normal,
82 mg “,, (normal
negative.
balls
ahc
because
body.
in these joints
E‘r;amination
Interphalangeal
IimitatIon
Laboratory
23. urinalysis
but
over the face. arms.
examinations
neck motion.
of the ankles. LSR
the
of renal.
have fine rales at the bates which
proximal
IS moderate
decreased
cholesterol factor
and
etTects.
very marked
was made
of her whole
joints
hands.
medication,
later.
to Involve
and over the past fete months
evidence
over
years
the halls of her feet for years.
floated
symptomatic and
of her ~a”
chest. neck. arid lower lips. The lung, Heart.
the maln
in the wrists
recedcnce
of the skin generally.
is prewnt
and under which
is ;I 52.!r-old
progressing
(PSS)
the skin
case appear\
patient
eventually
of scleroderma
She has no cllnlcal
At present,
She has minor
stools
This
The
at age 22. Fifteen but
involved
on her fingertips bulky
arthropathy.
arthropathy.
hands.
eventually
dysphagia.
and shoulders. problem.
and
1959. at ape 40. :L diagnosis which
resorptlve
phenomenon
the wrists
to live years ago she began noticing has experienced
and
resorptive
Raynaud’s
mainly
most of her joints. classical
scleroderma PSS with
of “pure”
right
negative. chungc\ telewwplng
for PSS: small pseudowcculacardiophrenic
ECG was normal.
Sjogren’.\ SJndrotne ISS/. It may appear somewhat artiticial to include Sjogren’s or the sicca syndrome as a separate form of resorptive arthropathy: however, it is included to emphasize the importance of its association with other disorders. The syndrome has long been recognized to occur with rheumatoid arthritis: however, its coexistence with PSS, SL.E, and polymyositis is less well appreciated.“~‘” ‘j Although Block et al.7J noted no difference in the severity ofjoint manifestations of patients with or without the sicca syndrome. live of our 35 patients with rheumatoid arthritis and the sicca syndrome observed prior to our study of resorptive arthropathq were found to have resorptive arthropathy, an incidence of resorptive arthropathy much higher than is expected in rheumatoid arthritis without the sicca syndrome.“.” It therefore seems doubly striking that the coexistence of Sjogren’s syndrome and resorptite arthropathy has been reported but twice. 2”,77Although Mather’s”’ patient had typical rheumatoid arthritis, the patient reported by Rogers and Lansbury” had the sicca syndrome associated with polychondritis. This is the only patient Mith the very rare polychondritis and resorptive arthropathy that we have been able to discover. Mulricentric
Rrticulohi.Ftioc??ro.Fis (Lil,oid-L)ertllrIfo-if
rthritisj
M ulticentric reticulohistiocytosis (M R) is a rare disorder single to numerous brown to yellow papular tnucocutaneous
characterized bq lesions hith ;I
214
SWEZEY
ET AL.
predilection for location on hands, arms, face, and neck.78m8”The lesions contain numerous large lipoid laden histiocytes which stain poorly with Sudan III and IV, but strongly positive with Sudan Black B and the periodic acid Schiff technique.7n The disorder may be confined to the skin, but it is often associated with a generalized acute and chronic destructive arthropathy in which synovial tissue has been shown to reveal the same histocytic changes.78~80 In a review of 23 cases, three-fourths occurred in women and essentially all ages and races have been represented.78,80~x2 One fifth of these patients had resorptive arthropathy.80,82 The distinguishing radiographic features of M R are more rapid bone than artitular destruction; extensive terminal interphalangeal involvement; and unusual sites of involvement including lumbar facet joints, cervical spine including the odontoid process, costotranverse joints as well as connective tissue attachments on the femoral trochanters, ischial tuberosities, anterior angles of vertebral bodies, and the shafts of Long bone such as the radius and ulna.7’~80~x3~84 The location of the skin nodules over the joints mimicking rheumatoid nodules may greatly add to the difficulty of distinguishing this entity. In addition to the radiographic features, the absence of rheumatoid factor, the not infrequent association of hypercholesterolemia and xanthelasma, and above all, a positive skin lesion or synovial biopsy serve to differentiate this unique disorder.78,7’ DIFFERENTIAL
DIAGNOSIS
Neuropathic Joints Although the neuropathic joint clinically and radiologically may be indistinguishable from a resorptive arthropathy, the neuropathic joint is invariably found in diseases that manifest impaired deep pain and proprioceptive sensations. The neuropathic or Charcot joint typically is characterized clinically by its remarkable instability, relative painlessness, and usually the presence of a marked effusion. Pathologically, with the exception of synovial chondromatosis, the presence of intrasynovial bone and cartilage fragments is unique.” Radiographically, the counterparts of clinically observed joint hypermobility, effusion, resorption of articular surfaces, fragmentation of articular bone, profuse disorganized hypertrophic articular and extraarticular bone proliferation are all features of the neuropathic joint.85 ” The two disorders most commonly associated with the Charcot joint are tertiary syphilis (tabes dorsalis) and diabetes mellitus (posterior column sclerosis and peripheral neuropathy).86,“8,89 Neuropathic joints are much less common in the upper extremity than in the lower, and when they occur in the upper extremities, they are usually found in 9om92 Tertiary syphilis, cervical ribs, association with leprosy and syringomyelia. and multiple sclerosis have been reported as causes of upper extremity neuropathic joints, and it is of interest that only one example of a Charcot joint occurring in the upper extremity (a wrist) has been reported with diabetes mellitus.” K9mY4 Charcot joints hdve been described in a number of other disorders including congenital insensitivity to pain, peripheral neuritis, spinal cord tumors, spinal
OPERA-GLASS
215
HAND
cord trauma or myelitis, malnutrition, and pernicious anemia with subacute combined degeneration of the spinal cord.95m98 The extremely rapid progression of some of the cases of Charcot joints (as little as nine days with associated sepsis and 446 wk in the absence of known coexistent infection) compares favorably with the most rapid recorded development of primary resorptive arthropathy, 4 mo as reported by Baker.32,87,92,94 Osteomyelitis is a frequent complication of the neuropathic joint.“‘~“‘.” It is particularly apt to occur in the fingers and in the metatarsal and phalangeal bones adjacent to the soles of the feet. 9o In syringomyelia and Hansen’s disease infection is frequently seen in association with resorption of the phalangeal tips of the hands.64 A number of other disorders recorded as producing similar digital tip resorption, although not necessarily on a neuropathic basis, include in addition to scleroderma, diabetes mellitus, syphilis, ainhum. bronchiectasis. Burger’s disease, hematoporphyria, polyvinyl-chloride exposure. and Ra!,naud’s disease.64m69 Thus, resorptive arthropathy can be distinguished clinically from neuropathic joint disease by the type of associated arthritic disorder, the lack of neurological manifestations and the lack of infection complicating the joint destruction. Pathologically, the lack of identifiable synovium in resorptive arthropathy contrasts with the intrasynovial bone and cartilage fragments that disiingulch the neuropathic joint.4.15.‘8.21,85 Casr 10:
(M.W.)
black female
with
(Fig. a 20-yr
19) Syringomyelia history
history of loss of temperature
and digital tuft resorption
of repeated
perception
burns and infections
in both hands and forearms.
The patient of both Over
is a 36.vr-old
hands and a Ii!-kr this permd
01 tune
her hands have become thick and fleshy. She has had a cellulitis of the right index finger in 1464. the left second and third fingers in 1964. the left hand in 1965. and the left middle finger I” 1967 Examination
in 1965 showed findings compatible
and a diagnosis compatible
Fig. 19. tissue langes, bone
Case
and
degenerative joint
are typical
disease.
10. Syringomyelia. resorption
of
the
arthritis
of the thumb of the changes
with a spinal cord lesion involving
was made. A myelogram
with a diagnosis of syringomyelia
swelling,
phalangeal
of syringobulbia
and
Diffuse distal of
the
ectopic
soft phainternew
in a neuropathic
was performed
C5- T9 or 10
and the findlnpa
were
216
SWEZEY
Fig.
20.
neuropathy foot.
Case has
Extensive
11.
normal severe a
of
the bony
joints toes
neuropathic
of bone and
condition from
Alcoholic
at
the
metadisloca-
Maintenance in the
arthropathy
picture
AL.
neuropathic
subsequent
present.
mineralization
resorptive
radiographic
is
foot.
in a typical
resorption
tarsophalangeal tion
Neuropathic
resulted
ET
presence
is characteristic and
distinguishes
rheumatoid
of of
a of
the
arthritis.
neuropathy, secondary osteomyelitis. and bone destrucCase II: (S.C.) (Fig. 20) Alcoholic tion. The patient is a 49.yr-old black male with a past history of chronic alcoholism with secondary peripheral neuropathy and a seizure disorder. He has a history of bilateral foot osteomyelitis dating back to 1941 with intermittent drainage from both feet over the years with variable Klebsiella, and Aerobacter organisms. He has no past history of positive cultures for Proteus, diabetes mellitus and a recent VDRL was negative. In 1963 his tifth toe was amputated due to the osteomyelitis. In November 1969 examination revealed draining sinuses in the left foot with stasis dermatitis, increased pigmentation. and swelling of the feet bilaterally. In April 1970 he developed a squamous cell carcinoma in the floor of his mouth. Case I2: (F.M.) (Fig. 21) Diabetic neuropathy, plantar ulceration, and secondary osteomyelitis. The patient is a 54-yr-old white male who had the onset of diabetes mellitus in 1950 at age 37. The diabetes required treatment with insulin (NPH-20-30 units per day) for the first 14
Fig.
21.
changes langeal
Case of
the
metatarsal osteomyelitis multilated
12.
Osteomyelitis.
metatarsal
bones
neuropathic ever,
the
are
and shaggy
similar psoriatic
periosteal
bones as extremity.
to
proximal the
pha-
changes
arthropathies. new
suggests the
Resorptive
and
underlying
the
bone
along
presence cause
of How-
of
the of this
OPERA-GLASS
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Fig. 22. Case 13. Leprosy. It is rare for a neuropathic condition to be unaccompanied by infection when it occurs in the foot. The resorptive changes that are seen therefore may be due either to the primary neuropathic problem or the coexistent infection. Plantar ulcers were present beneath the fourth and fifth proximal phalanges and appear to have accelerated the resorptive changes. Additional resorption is seen in the distal phalanges of the second, third, and fifth toes.
yr of the disease and was controlled with diet alone after that. He has a significant history of excessive alcohol intake since 1943, requiring numerous admissions to hospital for delirium tremens. In 1957 he complained of burning of the soles of his feet and on examination jn 1959 was found to have decreased sensation to pinprick in the legs from mid-thigh down to the toes. He has been noted to have decreased arterial pulses in his feet since 195X. In I960 he developed osteomyelitis in the left foot secondary to an infected plantar ulcer and in 1963 he burned the soles of both feet on hot asphalt and subsequently developed chronic plantar ulcerations on both feet with btlateral osteomyelitis. In April 1967 he had bilateral above-ankle amputations as treatment ol osteomyelitis. Pathologic examination of the feet showed arteriosclerosis obliterans changes III the blood vessels. Of significance in the past history is intermittent hypertension since 1940. HIS father, brother, and sister have diabetes mellitus. Significant physical flndings other than the findings in the legs were mild hypertensive arteriolar findings m the vessels of the eyes. Lahoratory tests show elevated blood sugar, evidence of liver disease (BSP 26”,, at 30 min). and nonspecific ST changes on ECG. Case 13: (M.S.) (Fig. 22) Leprosy with neuropathy. The diagnosis of leprosy was made in thts 35.yr-old white female in 1961. She presented symptoms of decreased sensation and joint swelltng in the lower extremities. Examination demonstrated many ulcerations over the legs, a spotty dccreased sensation over the calves. decreased sensation over the area of the distal ulna with atrophy of the hypothenar eminence of the left hand, swelling of the distal phalanges of both fifth fingers, and hepatosplenomegaly. Liver biopsy showed multiple granulomata without necrosis or giant cells. Acid fast organisms were demonstrated. Skin biopsy &as characteristic of lepromatous leprosy
Erosive Osteoarthritis
Erosive osteoarthritis (EOA) is the most recent arthropathy to be identified in the group of disorders associated with resorptive arthropathy. In two separate papers in 1966, Peter et al.‘“‘~“” called attention to a particularly aggressive, destructive form of primary osteoarthritis, which by virtue of an inflammatory synovitis caused marked erosion of distal and proximal interphalangeal joints. One of the patients (Case 5) reported by Peter, Pearson, and Marmor’“O demonstrated destructive changes compatible with resorptive arthropathy. but none of these cases manifested the resorption of the adjacent bone. C‘UW14: (R-B.) (Fig. 23) Erosive arthritis with severe distal and proximal interphalangeal
SWEZEY
ET AL.
Fig. 23. Case 14. Erosive osteoarthritis. The typical distribution of changes in erosive osteoarthritis are present in both hands, involving the distal and proximal interphalangeal joints, and the trapeziometacarpal areas. The erosions and subluxations are so extensive that a wavy contour of the fingers mimics the appearance of an “opera glass” hand.
joint destruction. The patient is a 61-yr-old white housewife with progressive distal interphalangeal and proximal interphalangeal arthritis of at least 5 yr duration, Aside from trapezoimetacarpal hyperthrophic changes, no other joints were involved. There was no prior history of arthritis. There was no evidence of psoriasis or systemic disease. No intraarticular steroids bad been administered. rheumatoid factor
Sensation and determinations,
motor and
function serum
were intact. The CBC, sedimentation uric acid were all within normal
rate, limits.
The hands demonstrated marked digital deformity with hypermobile interphalangeal joints and some redundancy of overlying skin. There was only slight tenderness of the affected joints. Radiographs demonstrated extensive distal and proximal interphalangeal joint erosion and subluxations with hypertrophic changes in the trapeziometacarpal joints. This case is presented as an example of erosive osteoarthritis and severe joint destruction without actual bone shaft resorption.
A vascular Necrosis
Avascular necrosis occurs insidiously in the absence of any associated arthritis. It is extremely rare in the small joints of the hands. It is best distinguished from resorptive arthropathy radiographically early in the course by the lack of joint margin erosion and by the destructive changes limited to only one articular surface. Later when both joint surfaces are involved, the joint space is narrow and secondary proliferative and degenerative changes predominate. Avascular necrosis of bone occurs when an area of bone has been deprived of its blood supply by any means, excluding infection. The areas most frequently involved are those with a tenuous blood supply and poor collateral circulation, such as occur in the epiphyseal areas. In the adult, common epiphyseal locations affected are the head of the femur, the humeral head, distal femur, and proximal tibia. Less common locations are the distal tibia, body of the talus, carpal scaphoid and lunate (common after trauma), and the humeral involved. In the juvenile capitellum. lo3Two or more areas can be simultaneously with a developing skeleton, other areas involved are the vertebral bodies, tibia1 tuberosity, patella, OS calcis, and head of the metatarsal.“* Shawl” described a case involving the phalanges of the hands (Thiemann’s disease); however, a destructive arthritis did not result. The earliest X-ray finding is an area of arclike radiolucency paralleling the (a relative or subchondral surface of the involved bone. lo4 Later, osteosclerosis absolute increase in the X-ray density) of the affected bone end occurs and
OPERA-GLASS
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cystic and sclerotic islands appear in the resulting, often wedge shaped, infarct of bone.“” Continued stress and weight bearing produces secondary subchondral fractures in the necrotic and newly formed bone resulting in fragmentation and collapse of the bone with irregularity of the joint margin, destruction of the cartilage, and prominent secondary degenerative joint changes. Avascular necrosis has been reported to occur in association with many disease states. They include hemoglobinopathies (mainly sickle cell disease and sickle cell trait, hemoglobin SC disease, and hemoglobin S-Thal disease). caisson disease, dislocations and fractures of the hip, SLE, giant cell arteritis, pancreatitis, alcoholism, Gaucher’s disease, Cushing’s disease, steroid usage (systemic or intra-articular). hyperuricemia and gout, and renal transplantation (probably due to associated steroid treatment).‘“’ ‘I6 Although the exact mechanism of production of avascular necrosis by all of the foregoing is not known, the end result is the obliteration of the blood supply to a particular area of bone. Avascular necrosis does not commonly produce a picture one would confuse with resorptive arthropathy. Typically, it is the weight-bearing joints that are involved, and from a review of the literature, it appears that when multiple joints, including non-weight-bearing joints are involved patients are usually on high steroid dosages, e.g., renal transplantation patients.“b One such case had a total of 10 separate joints involved.“’ Uncommonly, the small bones of the hand are involved and in those cases, resorption is not a prominent hnding.“h The case we have presented (A.S.) is unusual in the number of joints involved and the severe destructive process incurred.
Fig. 24. Case 15. Avascular necrosis. Chronic high dosage of adrenocorticosteroids in a patient with systemic lupus erythematosus has resulted in avascular necrosis of both shoulders and hips. Loss of the humeral heed and diffuse osteoporosis are present.
SWEZEY
ET
AL.
Fig. 25. Case 15. Avascular necrosis. Similar changes of the femur have caused marked resorption of the femoral head.
Case 15: (AS.) (Figs. 24 and 25) Avascular necrosis of multiple joints in a patient with systemic lupus erythematosis on high dosages of steroids. The patient is a 35-yr-old white female whose history dates back to 1962 at which time she had the onset of polyarthritis associated with pneumonitis, anemia, and hair loss. A diagnosis of SLE was made on the basis of positive LE cell preparation and compatible clinical findings and she was started in high dose steroid therapy, at times as high as 200 mg/day. She was on a very high dose of steroids for several months at which time she was first seen in our clinic. She was gradually tapered to a maintenance dose of 4-8 mg methylprednisone a day. Over the years she has had no severe manifestations of her disease except for intermittent increases in arthralgias. There has never been any evidence of renal disease. During a pregnancy in 1964 she began complaining of increasing pain in her shoulders, hips, and knees. and over the ensuing months, a diagnosis of avascular necrosis in each of these joints was made. In April 1970 she developed a Staphylococcus mucus infection in her right hip joint. Later, pseudomonas organisms were cultured from the same area. In August 1970, a Girdlestone operation was performed on the right hip. However, she then developed difficulty voiding and woke associated one morning with a tingling sensation from her waist down to her toes bilaterally, with increased weakness and numbness in her lower extremities. An emergency laminectomy from T5 to T9 was performed, and she was found to have a collapsed seventh dorsal vertebra, presumably the result of severe osteoporosis, which was compressing the spinal cord. Postoperatively, she very slowly regained strength and sensation in her legs; however, recovery was not complete. Past history includes a bleeding duodenal ulcer in 1964 and 1969 and a left tibia] osteotomy in November 1969. A cousin has rheumatoid arthritis and an uncle may have scleroderma. On physical examination she is a chronically ill appearing woman. On examination of her heart, she has grade II/VI systolic murmur at the base without radiation. Pertinent physical findings are otherwise limited to her musculoskeletal system. The shoulders are limited to 90” and she lacks IO”--15” extension on the left. The right hip has the residuals of the Girdlestone procedure. The right knee has normal motion, but the left knee is posteriorly subluxed and has a range of motion from 25” I IO” with significant pain on motion. She is incapable of ambulation. In
OPERA-GLASS
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January 1972, on examination, she had generalized muscle weakness, more pronounced in the lower extremities; however, sensory examination and tendon reflexes had returned to normal. Laboratory tests: Hb. 8.5, WBC 11,000 (normal differential), platelets normal, urinalysis showed many WBCs without significant bacteriuria. She has never had significant RBCs or casts in the urine and 24-hr urine studies have never shown significant proteinuria. Other test results are: BUN 20, LE cell preparation positive, anti-DNP antibody positive undiluted, anti-DNA antibody negative serum complement normal, rheumatoid factor negative, ECG normal, chest X ray normal.
Sickle
Cell Disease
Sickle cell disease in the adult is characterized by avascular necrosis and large joint involvement. Osteomyelitis is not an uncommon complication in both children and adults. In young children, hands and feet may be involved in a disorder distinguished from resorptive arthropathy by the presence of periosteal proliferation, and occurring in an age group in which resorptive arthropathy has not been seen. Osseous involvement in sickle cell disease is estimated to occur in up to 65”,, of patients.“’ Changes have been reported not only in homozygous SS disease but also in SA, SC, and S thalassemia.“8 The basic pathogenesis in all cases is vascular occlusion secondary to the sickling phenomenon initiated by deoxygenation of the blood. Those areas of the skeleton with the poorest primary and collateral circulation are predisposed to the most severe and frequent involvement. Skeletal involvement associated with sickle cell phenomena can be divided into the following three groups. This is the most common bony manifestation of (1) Hand-Foot Syndrome: ‘I9 in a series of 1200 patients in the disease in children.“’ Lambolle-Legrand, Africa, states that over 80”/, of the patients exhibited the hand-foot syndrome during their infancy and childhood. The condition occurs in repeated attacks from age 6 to 24 and is very rare past the age of 6 yr.‘18 Prior to the sixth year, the small bones of the hands and feet contain red marrow. After the age of 6 yr, the red marrow recedes from the small bones leaving less vascularity and less opportunity for sickling. Clinically, the affected children have swelling and pain of the hands, feet or both. The swelling and pain last up to 2 wk and then subside. The hands are frequently symmetrically involved and occasionally all four extremities are involved at the same time. X-ray findings show diaphyseal and metaphyseal periosteal elevation, subperiosteal new bone formation, and a moth-eaten appearance to the shaft.12’ The joints are not directly involved and usually all the bone changes are completely reversible.‘i8 Occasionally the bone changes are very destructive and Watson et al.“’ reported the complete disappearance of a metatarsal. (2) Avascular Necrosis: Golding et al.“* report a lO”/,)- 12:;) incidence of avascular necrosis in the adult. Hemoglobin SC was involved more commonly than hemoglobin SS disease. The findings are as described in a previous section on avascular necrosis. No cases with a marked resorptive quality have been reported. Osteomyelitis is a relatively frequent complication of hemo(3) Infection: globin SS disease. The most common infecting organism is Salmonella, although involvement by Staphylococcus, Streptococcus, Shigella, and E. coli have been reported.‘23,‘2’ It occurs more frequently in children thus making the
222
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differentiation from the hand -foot syndrome very difficult. Constant et al.‘23 reported a Salmonella osteomyelitis with total destruction of a proximal phalynx of a thumb and a middle phalynx of a finger. Rubin et al.‘24 reported a case of Shigella osteomyelitis with extensive destruction of metacarpals, metatarsals, and phalanges in a 23-yr-old patient. The osteomyelitis rarely involves the joint space and thus a very destructive bony process may be present without the joint features characteristic of a resorptive arthropathy. Steroid
Arthropathy
In addition to avascular necrosis induced by systemic steroid therapy, a Charcot-like arthropathy has been reported from the use of intraarticular steroids.‘25-‘29 These changes have been observed thus far primarily in the knees and hips with both local and systemic steroid therapy.‘25m’30 In only one patient (a 43-yr-old woman with osteoarthritis who allegedly received a minimum of 200 injections into each interphalangeal joint in both hands over a 3-yr period) has a steroid related resorptive arthropathy in interphalangeal joints been reported, and the possibility remains that this patient may have had erosive osteoarthritis.‘30 In most cases, pain relief with steroid therapy appeared to permit joint abuse and joint destruction.‘25 ‘29 The experimental demonstration of cartilage damage in rabbits receiving two or more weekly intraarticular knee injections supports the local destructive propensity of the steroid crystals.13’ Differentiation of this complication of steroid therapy from resorptive arthritis should not pose a significant problem because of the restriction of the steroid induced arthropathy to those joints receiving multiple injections. Further, resorption with remodeling of the shafts of the bones adjacent to the joints has not been seen. Gout
Chronic tophaceous gout may result in a mutilating arthritis, with erosion of bone from adjacent soft tissue masses. In addition to pressure erosions, intraosseous tophaceous deposits contribute to the bony destruction.‘32 Occasionally, avascular necrosis contributes to the dissolution of bone.‘i5 Distinction from the classic forms or resorptive arthropathies is evident both by the appearance of the soft tissue and the character of the erosions. In chronic tophaceous gout, soft tissue masses deform the soft tissues but do not cause a redundancy of skin. They may contain calcium.‘32*‘33 Their contiguity to the bony erosions is often apparent. Erosions tend to be periarticular and have a characteristic overhanging edge and sclerotic border, in contrast to the whittled resorption and the absence of productive new bone of the resorptive ar‘34Progression of the periarticular erosion to complete destruction thropathies. of the joint may occur and replacement of the cartilage by fibrous tissue may mimic the changes described in the classic main en lorgnette hand and foot. In chronic tophaceous gout, multiple joints are frequently involved and at times the radiographic picture is indistinguishable from rheumatoid arthritis.13* However, the similarity is in the well-circumscribed cystic changes of the hands and feet, and gout never mimics the bone shaft remodeling of resorptive arthropathy.
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Fig. 26. Case 16. Gout. Mutilating arthritis from chronic tophaceous gout may cause severe destruction of the adjacent bones. This is most evident at the distal ulna and a large erosion with an associated tophus is present. Chronic draining tophi may ultimately lead to amputation as seen in the fifth finger, contributing to the mutilating effect of gout.
Recently, an enzyme defect as etiologic factor of hyperuricemia has been discovered and described clinically as the Lesch-Nyhan syndrome. The syndrome incorporates hyperuricemia, occasionally acute gout, mental deficiency, choreoathetosis, and a propensity for self-mutilation, such as chewing off the finger tips. Primary destructive joint disease has not been described although tophi can be present.13’ C‘ase16: (F.M.) (Fig, 26) Severe generalized tophaceous gout with marked destructive changes simulating resorptive arthropathy. The patient is a 25-yr-old white male who has had acute attacks of pain, heat, redness, and swelling of the joints, recurring at intervals since age 13. The attacks have occurred in almost every joint in the body. He had repeated admissions for excisions of tophi. amputation of toes, and later a lower leg amputation. He was generally stiff and had very painful joints throughout the body. His spine was nearly ankylozed. Numerous tophi, notably along the anterior tibia1 and forearm regions were present. Blood pressure 180/124, LAB. NPN 20 mg%. uric acid 0.6”/,-12.4 mg% urinary uric acid excretion-over 2 g/day: PSP test--60”/” excretion in I hr; S.G. 1.016; Hinton negative. Radiographs revealed extensive soft tissue tophi and generalized joint destruction secondary to tophaceous deposits. Sarcoidosis
Sarcoidosis not uncommonly affects the bones and joints, each being involved in as many as 1.5):;,-207,, of cases. The arthritis produced by sarcoidosis may be acute (and often associated with erythema nodosum) or chronic. Neither type commonly results in joint destruction.‘37 ‘39 Bone involvement is seen radiographically as a diffuse osteoporosis, a resorptive whittling, a lacelike coarse trabecular pattern, or as punched out cystic lesions.‘40 Joint destruction has been reported to occur as an extension of the cystic lesions from the bone into the ankle joint and the authors have observed such a case involving the knee joint.‘4’m’42 Neither case could be confused with a resorptive arthropathy. Case IX demonstrates very extensive bone involvement by sarcoidosis in the hands: however, the joints are not involved. Case 17: (M.G.) (Fig. 27) Sarcoidosis with cystic and resorptive changes. The patient is an 8O-y/r-old black female in whom the diagnosis of sarcoidosis was established at age 55. Initially,
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Fig. 27. Case 17. Sarcoid. Although relatively rare, resorption of bone in patients with sarcoidosis results in a whittled appearance of the phalanges as is seen in the third finger, in addition to the more typical defects in the phalanges of the third and fourth fingers. The coarsened trabecular pattern of the middle phalanx of the second finger is the third bone pattern seen with sarcoid and implies diffuse involvement by noncavitating granulomas.
involvement was mainly cutaneous. At one time a severe lesion in the nasal septum necessitated the use of Prednisone, 20 mg/day. Bilateral hilar adenopathy has been present since the onset: however, she has had no other parenchymal involvement. Progressive involvement of the bones of her fingers and wrists are illustrated. In January 1971 she developed nodules on her vocal cords shown to be sarcoidosis on biopsy. Skin biopsies have all been consistent with sarcoidosis. Case 18: (I.H.) (Fig. 28) Sarcoidosis with marked cystic and lacelike destructive changes in the phalanges. The patient is a 42-yr-old black female who was first seen at age 39 because of painful hands. This problem became progressive, and she was observed to have severe destructive changes in her phalanges. Her chest radiograph demonstrated prominent hilar adenopathy without other abnormalities. Her PPD #I was negative and her serum uric acid normal. Details beyond this summary are not available as her record has been lost and the patient has not been seen since 1955.
Tuberculosis
Tuberculosis only rarely involves multiple small bones, and when secondary joint destruction occurs, at most a few joints are affected. Like sickle cell
Fig. 28. Case 18. Sarcoid. Diffuse involvement of the spongiosa may occasionally destroy the bone so extensively as to cause a radiographic picture simulating resorptive arthropathy. However, this is easily distinguished from the classic causes of resorptive arthropathies by the location of involvement. The process has destroyed the diaphyses of the middle and distal phalanges, but spared the articulating ends of the bones so that the joints are relatively normal.
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disease, the age group in which hands or feet may be diffusely involved is much younger than that of resorptive arthropathy which is a postadolescent disorder. Skeletal tuberculosis is probably the most common form of extrapulmonary tuberculosis today. Of 100 patients seen at the State University of Iowa Hospitals with extrapulmonary tuberculosis from 195551966, 35 had skeletal involvement, 20 lymphatic, and 19 renal. ‘43 The basic lesion in skeletal involvement is usually a combination of osteomyelitis and arthritis and in adults the weightbearing joints are most commonly involved. Davidson and Horowitz’43 give the following incidence of involvement: spine 50%; hip 15%; knee 1.5%; wrist, elbow, shoulder, other bones 20”;,. The juvenile age group has a much higher incidence of involvement of the small bones of the hands and feet and more commonly has multiple bone involvement.‘44 Tuberculous bone involvement can be divided into localized and disseminated forms. The localized form is a combination of osteomyelitis and septic arthritis and usually involves only one joint, but more rarely a series of joints (as in tuberculous vertebral osteomyelitis) may be involved. With chronicity the infection can result in marked bone destruction and restriction of joint motion.‘45 Kelley and Karlson’4h reported destructive processes involving the wrist and ankle joints, and others have reported involvement of the small bones and joints of the hands and feet with associated destruction of a phalynx or metacarpal.‘47~‘49 Although the cases reported have destructive changes, they are usually localized to one or two joints and do not have the diffuse resorptive quality of the classic resorptive arthropathies. The disseminated form of osseous tuberculosis is uncommon and occurs mainly in young children and young adults.‘jO Typically in tuberculous dactylitis, the peripheral tubular bones are expanded and involved with numerous cystlike lesions.‘s’ The joints themselves are rarely involved and thus this entity should be readily excluded in the ditferential diagnosis of a resorptive arthropathy. Recently the atypical mycobacteria have been receiving more interest, and joint involvement due to one of these organisms has been reported. Klienenberg et al.“’ reported a case of destructive arthritis involving the proximal and distal interphalangeal joints apparently due to a photochromogen. None of these joints in their case demonstrated the classic resorptive arthropathic changes.
Extensive resorption of joint surfaces is not a factor of hyperparathyroidism. The primary lesion of hyperparathryoidism is found in the bone. The incidence of bone changes on X ray in patients with hyperparathyroidism ranges from 20”,, to 30% in various series.‘j3,‘j4 The findings include subperiosteal resorption, osteoporosis, disappearance of the lamina dura about the teeth, resorption of the distal ends of the clavicles, loss of height and biconcavity of the vertebra, and the late findings of bone cysts and brown tumors. Actual joint involvement in hyperparathyroidism can occur by the following mechanisms: ( I ) Calcium pyrophosphate dihydrate (CPPD) crystal deposition disease,‘s’,‘5h (2) gout,‘j7.‘5K (3) periarticular calcification with inflammation,‘5y and (4) a condition described by Bywaters I60 in 1963 which can mimic rheumatoid
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Fig. 29. Case 19. Hyperparathyroidism. Extensive resorption of bone in hyperparathyroidism may rarely cause shortening of the phalanges and collapse of the overlying soft tissues. resulting in a clubbed shape to the distal fingers. The typical subperiosteal resorption alters the sharp outer contour of the bone in contrast to the changes of resorptive arthropathies. As in sarcoidosis, the joints are spared and the diaphyses are destroyed, further distinguishing the radiographic picture from the connective tissue disorders.
arthritis with joint inflammation and actual erosions in the joints on X ray. Bywaters believes the erosions are due to subchondral bone microfractures which result in dissolution of the hyaline cartilage. Case 6 in his series developed a complete collapse of the first metacarpal presumably from this process. Although erosions and minor resorption of the joints do occur, there have been no reported cases in the literature to date that could be confused with a resorptive arthropathy. The case of resorptive arthropathy reported by Nelson in 1938 was associated with a parathyroid adenoma, however, there was no clinical evidence of hyperparathyroidism.” Case 19: (C.J.) (Fig. 29) Primary hyperparathyroidism, secondary renal failure, and severe bone resorption. The patient was a 56-yr-old white male when he first presented to the LAC/USC Medical Center in June 1962 with azotemia. In 1946 he passed a single renal stone. He had a several-year history of polydypsia and polyuria; a 445-yr history of arthritis, specifically involving the left ankle and which had been diagnosed but not documented as gout in the past; general aching in the extremities for years; clubbing of the distal phalanges for years; shortening of stature and kyphosis; and the onset of nausea, vomiting, and uremia in May 1962. In June 1962 he had a calcium of 10.0, phosphorus of 4.7, and X rays of the bones were consistent with far advanced hyperparathyroidism. The patient’s uremia progressed and on November 8, 1962 surgery was performed and a parathyroid adenoma removed. The other three parathyroid glands were atrophic. Preoperative laboratory tests were: Hb. 9.7, WBC 7700, ESR 64, Ca 10.4, Ph 6.9, uric acid 10.8, Creatine 6.2, K 3.5, Na 137. Post-operatively, he developed hypocalcemic tetany. He died on February 4, 1963, of progressive uremia.
Biliary Cirrhosis
A destructive process involving the joints similar to hyperparathyroidism has been described in biliary cirrhosis with hypercholesterolemia.‘6’ The erosions in these cases were not severe and resorption was not present. In both of these diseases the mechanism of erosion is extra-articular in contrast to the primary intra-articular process of a resorptive arthropathy. Osteolysis
Although massive bone resorption characterizes the conditions that cause osteolysis, absence of primary joint involvement serves to distinguish these entities from resorptive arthropathy. Hemangioma: There are two major forms of osteolysis. The most dramatic
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dramatic form, massive osteolysis, was characterized by Gorham16* as a disorder primarily affecting children or young adults caused by a “benign” hemangioma. Multiple contiguous bones may be progressively involved, but the disease is usually self-limiting. ‘62m’b4The pelvic and shoulder girdles are the usual sites of involvement. Typically endosteal new bone is laid down as the periosteal cortex adjacent to the angiomatoses is resorbed. This gives the appearance of concentric shrinkage and tapering of the affected bone until the two cortices meet, merge, and disappear.‘64 Familial Osteolysis: Familial osteolysis comprises the second category and consists of adult and juvenile forms. Acre-osteolysis is a dominant disorder of adult life characterized by disintegration of bone of the phalangeal tips. but also involving metacarpals and metatarsals in a more inflammatory necrotizing osteolysis. Sensory changes are absent, but joint hypermobility and congenital anomalies of the skull and spine are frequently seen and secondary osteomyelitis of the phalanges is a common complication.“’ Essential osteolysis with nephropathy is another dominantly inherited disorder affecting children at age 3 and 4 yr with an active arthritis typically involving the carpals and tarsals. ‘b6,‘b7The process becomes quiescent and then late in the third decade, terminates with collapse and lysis of the affected bones and their partial extrusion through the skin over the volar surfaces of the involved joints. In some patients, the process is limited to one extremity and in others, elbows and a clavicle have been involved.‘6h,‘67 In about half of the patients, there is an associated slowly progressive glomerulonephritis with hypertension and ultimately death from renal failure.“’ Biopsies of synovia have revealed only proliferation and hypertrophy of smooth muscle cells in arterioles and some areas of dense collagen tissue and lakes of metachromatic staining. “’ Bone biopsy revealed areas of cortical bone. Four renal biopsies showed eosinophilic tubular precipitates while one of these showed moderate arteriolar sclerosis. ‘66 The pathogenesis of the resorptive phenomenon cannot be elucidated from these findings. The familial nature of this disorder and the lack of involvement of the interphalangeal joints serves to readily distinguish it from resorptive arthropathy. A self-limited osteolysis following trauma has been reported.‘6x One case involved a wrist, and the second, a distal clavicle. No biopsies were available from these cases, and it is therefore impossible to classify them further.lhx Werner’s
Syndrome
Werner’s syndrome is a Mendelian recessive disorder characterized by an adult onset of premature aging. ‘69+‘72Subcutaneous calcification, sclerodactyly associated with atrophic skin, and resorption of the distal phalanges cause this rare entity to be confused with progressive systemic sclerosis. The predominance of skin and skeletal involvement in the feet and relative sparing of the upper extremities should suggest a separate entity as this pattern of skin and extremity involvement is the reverse of scleroderma. Keratomas and indolent ulcers are commonly present on the soles of the feet, and skin ulceration with secondary osteomyelitis of the adjacent bone complicates the picture of resorptive changes.lhy ‘72
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Additional features of Werner’s syndrome differentiate it from scleroderma: premature aging with early cessation of menstruation in the female; arrest of growth at puberty; premature balding; juvenile cataracts; hypogonadism; osteoporosis; osteoarthritis; arteriosclerosis; and diabetes. Clinically, the patient has a birdlike face with beaking of the nose and loss of the periorbital connective tissue, short stature with spindly extremities and a weak, high-pitched, squeaky voice. Because these patients have a high incidence of malignant disease with a special predilection for the development of sarcomas, their distinction from scleroderma is important.‘69~‘72 Case 20: syndrome with severe bone demineralization and degenera(E.J.) (Fig. 30) Werner’s tive changes in joints and soft tissue in the feet. The patient is a 51.yr-old black female admitted to the LAC/USC Medical Center in October 1968 for evaluation of an ulcer in her right ankle, associated with pain and swelling of one month’s duration. A biopsy of this lesion revealed osteogenic sarcoma. She was seen by the Rheumatology Service because of intermittent pain and swelling of her wrists, metacarpophalangeal, proximal interphalangeal, knee, and ankle joints. Her hands and feet were the most severly involved. She had noticed these symptoms since 1962, and was bedridden for 2 mo in 1963. She had also noted thinning and graying of her hair for I2 yr, and cataracts of both eyes for many years. In the preceding 4 mo she developed anorexia, weight loss, and an exacerbation of her joint symptoms which had been quiescent since 1963. There is no family history of consanguinity. Her mother has diabetes mellitus, but her father is leg ulcers, and thinning of healthy. She has one brother with cataracts, small stature, diabetes, his skin over his lower extremities. Her sister and child are alive and well.
Past history revealed the patient to be gravida (toes were “shriveling up”) in 1957. right cataract repair in 1961. The patient was an alert female who appeared
2, para 2. She had a bilateral removal in 1959, and a right much
older
than
her stated
sympathectomy patellar tendon
age; blood
pressure
160/70, pulse 84 and regular, R 16, weight 100 Ibs. Her hair was fine, sparse and gray. There were scanty eyebrows and eyelashes. There was a beaked nose, recessed chin, and a small mouth. There was an aphakia-O.D. and a cataract-O.S. The fundi could not be visualized. There was a generalized lack of subcutaneous fat. The skin had punctate, dark brown lesions which did not blanch and some areas of hyper- and hypopigmentation. There was a Grade II ejection systolic murmur heard best at the base along the left sternal
Fig. 30. Case 20. Werner’s syndrome. Soft tissue calcification, resorption of the small bones of the hands, and subluxation of the distal phalanges have resulted in a picture of distal joint mutilation.
OPERA-GLASS
HAND
229
border. Neck. lungs, and abdomen were within normal limits. Peripheral pulses were normal. There was marked muscular wasting proximally and distally and firm masses were palpable in both triceps tendons and the olecranon bursa. Significant joint findings were: ulnar deviation; flexion contractures of the elbows, knees, and hips; marked atrophy of the skin over the lower extremities with marked atrophy and deformities of her toes; multiple areas of callous formation over the plantar surface of the feet; and a large swollen right ankle with ulcerating lesions. Neurologic examination was within normal limits. Hgb. 10.4, WBC 5700 (57-O. 28-14-I-10) with adequate platelets, slight anisocytosis and rouleaux formation, BUN 14, K 4.0, Na 132, Co2 28. PBI 3.5. T, test 12.7”,,, calcium 9.5. Pod 3.3. The alkaline phosphatase was 6.5, uric acid 6.4 mg %, total serum iron 25 pg %, IBC 243, and saturation lo”,,. Her GTT was abnormal (FBS 155 mg “4, I hr--185, 2 hr--193, 3 hr-I70 mg yO). serum complement 960 (normal), ANA negative, latex fixation negative, LE prep negative, antithymoglobulin titer l:l6. Serum proteins-A/G 3.8/5.2 with 1.6 beta and 2.3 gamma globulins. Radiographs revealed multiple soft tissue and vascular calcifications, degenerative joint disease in her hands and feet with severe demineralization in the bones of the feet, The right ankle revealed changes compatible with an osteogenic sarcoma. A bone and chest film were normal.
Involvement of multiple small joints in the hands by benign or malignant tumors has only been seen with multiple enchondromata. Although it is extremely rare for tumors to involve the bones of the hands and feet, this involvement does occur and can be classified as follows: (1) Soft tissue tumors. These include tumors of the skin, fat, blood vessels, lymphatics, nerves, and nerve sheaths. Usually they affect the bone only by compression, however, the more malignant tumors such as malignant hemangiosarcoma will occasionally metastasize to the bone producing destructive lesions. They usually do not involve the joints unless the tumor is highly destructive. 173Synovial sarcoma is a rare tumor which, although it does not usually arise from within the joint cavity, is capable of metastasizing to the joint and eroding it. ‘74 (2) Primary bone tumors. The solitary enchondroma, osteochondroma. solitary bone cyst, osteoid osteoma, aneurysmal bone cyst, and giant cell tumor all rarely involve more than one bone and do not cross the joint surface. Multiple enchondromata can produce a very destructive process involving the bones of the hand, however, the joints are generally spared in contrast to a resorptive arthropathy. “’ The more malignant tumors such as chondrosarcomas and osteogenic sarcomas also do not commonly produce a destructive joint lesion. (3) Metastatic tumors. Metastasis to the small bones of the hands and feet is a very rare occurrence,‘7h.‘77 a statement supported by Dolich et al. in 1970, who could report only 35 cases in their review of the literature.“” The incidence of primary tumor involvement in their series was: lung and nasopharynx, 50”,,; genitourinary system, 20”“; breast, lo”,); parotid gland, IO”,;,; colon, 5”,,; uterus, 5°,1. Multiple bones were involved in five of 23 case reported by Kerin,“’ and in his series only one metastasis involved a joint. Karten and Bartfeld,lx” in 1962, reported a case of bronchogenic carcinoma with extensive metastasis to the hands, who at autopsy was found to have diffuse metastasis in the distal and middle phalanges of a finger with tumor infiltration into the synovial membrane.
230
SWEZEY
ET AL.
Fig. 31. Case 21. Surgery. Surgical correction of dorsal contracted toes mimics the changes of resorptive arthropathies. Resection of the metatarsal heads and base of the proximal phalanges in a patient with rheumatoid arthritis has resulted in a tapered appearance of the bone and widening of the joint spaces.
It is therefore evident that tumors very rarely involve the bones of the hands or feet and even more rarely involve the joints to produce a destructive lesion. No cases have been reported that could be confused with resorptive arthropathy. Case 21: (M.G.) (Fig. 31) Surgical resection of the metatarsal heads for rheumatoid arthritis resembling resorptive arthropathy. The patient is a 63-yr-old white female with a 3-yr history of rheumatoid arthritis which has involved most of the joints of her body with persistent activity. The most severely affected joints have been her hands, both wrists, the left elbow, the right metatarsals, and cervical spine. Positive physical findings are restricted to her joints. She has swelling and flexion contractures of the proximal interphalangeal and metacarpophalangeal joints, subluxation and hammertoe deformities bilaterally, and plantar calluses on both feet. Laboratory studies include: Hb. 13.8, WBC 7800 (normal differential), ESR 26, urinalysis, normal; RA latex test reactive, and serum protein electrophoresis normal. Aspirin has been her only drug therapy. In March 1971 she underwent bilateral resection of the metatarsal heads with excellent postoperative functional results.
PATHOGENESIS
In most destructive arthropathies, such as gout, leprosy, infectious and rheumatoid arthritis, the cause of the joint destruction seems obvious. Why then has the pathogenesis of the severe resorptive arthropathies escaped satisfactory explanation? In attempting to relate a reasonable pathogenesis for a clinical syndrome that encompasses multiple diseases, we have assessed concepts that have been suggested by previous authors and integrated their speculations with documented data in an effort to achieve an intelligent understanding and working hypothesis for the genesis of these uniquely destructive arthropathies. A number of components encompassing inflammation, vasculitis, neural
OPERA-GLASS
231
HAND
lesions, bone lesions, and mechanical out steroid therapy will be considered.
factors
including
joint
abuse with or with-
Lack of Inflammation Although pathological data on resorptive arthritis have been collected since the report of Marie and L&i, there is essentially no new light shed on the question of pathogenesis of these remarkable deformities.4~9~‘2,‘5~‘82’.23 Specimens from these patients showed fatty and fibrous tissue replacing the articular structures and sparing the neurovascular tissues.4*9,‘S.‘9.2’.23 Although pannus is a typical feature of rheumatoid arthritis, it has not been found in those joints demonstrating resorptive changes, and in patients with resorptive arthropathy pannus is usually seen in those joint areas not manifesting resorption and otherwise typical of rheumatoid arthritis. Although erosive osteoarthritis does not represent a classical disease in the group of severe resorptive arthropathies, the spectrum of pathological changes from acute inflammation to fatty and fibrous replacement of articular structures described in this disorder may well be representative of what one might see in several distinct noninfectious entities associated with severe articular resorption. Peter, Pearson, and Marmor, lo2 in their study of erosive osteoarthritis, noted a spectrum of pathology ranging from synociocyte hypertrophy, lymphocyte and plasma cell aggregation and fibrin deposition to cartilage and bone replacement with fibrous tissue. The pathology was variable and tended to reflect the cliniNone of their patients had sensory cal intensity of the inflammatory process. impairment to suggest a superimposed neuropathic condition. In two of their cases, thickening of blood vessel walls was noted. There is no documented evidence of the pathological progression of resorptive arthritis, but there is radiological documentation of a typical rheumatoid films, it appears process progressing to the resorptive state. ‘* From sequential that the pathological process begins as an otherwise typical inflammatory erosion (as indeed was seen in the erosive osteoarthritis patient of Peter, Pearson, and Marmor) and ultimately reaches a state of severe destruction replacing the former articular structure as well as the inflammatory lesion that destroyed it.lX.102
Vascular Lesion Of what significance are vascular lesions to this disorder? Paradoxically, ischemia and hyperemia have both been correlated with bone resorption while sluggish but highly oxygenated blood flow is accompanied by osteoblastic activity.‘83 I’46Increased regional blood flow (hyperemia) in active rheumatoid arthritis has been demonstrated. ‘*’ Pathological evidence of a unique or fulminant occlusive ischemic vascular lesion in resorptive arthritis is lacking. 4,‘s~‘9*2’Vascular occlusive disease of the digital arteries as well as sympathetic hypertonia in rheumatoid arthritis both are sufficiently common that if either the subendothelial digital arterial lesions of RA or sympathetic hypertonia were a major factor in the genesis of resorption one would expect a greater prevalence of the resorptive phenomenon.i88~‘92
232
SWEZEY
ET AL.
The possibility that inflammation in rheumatoid arthritis disturbs the glomus body which controls metaphyseal circulation and causes increased bone resorption and an “opera-glass finger” has been suggested.ls4 Other acral vascular reflex disturbances are associated with pathological joint changes such as pulmonary osteoarthropathy and local gigantism. In this connection, JohnsonLs4 mentions examples of massive osteolysis (neuropathic joints) of the hips developing in 6 wk without obvious preceding trauma in patients at bed rest. He argues that this must be on a neurovascular basis and that these cases tend to refute the commonly accepted concept that repeated minor trauma with disturbed healing is the chief mechanism leading to changes in the neuropathic joint. The possibility of a neural defect leading to inappropriate shunting of blood by a disturbed glomus mechanism in neuropathic joints is given additional credence by the recent observations of Kiss et a1.‘93 who have shown arteriographic evidence of arteriovenous shunting in Charcot joints. Drennan et al. removed the synovia (the presumed area of shunting) in an attempt to improve bone circulation and enhance the prospect for successful fusions of neuropathic joints.‘94 Angiographic studies of digital vessels in rheumatoid arthritis have revealed hyperemia in areas of synovial proliferations, occlusive changes and hyperemic changes in areas of bone destruction and erosion, but no clear evidence of changes in the venous phase of the examination which might suggest abnormal shunting, thus mitigating against a disturbed glomus mechanism as the basis of the resorptive process.‘88~‘90~‘9’ If, indeed, a disturbed osseous vascular supply due to altered glomus function plays a role in resorptive arthropathy, it must be presumed, for reasons discussed below, to be on the basis of a local glomus disturbance caused by the arthritic process rather than a more proximal neuropathic process. Neural Lesion The similarity of the severe destructive nature of the lesions in resorptive arthritis to the changes in the Charcot joints raises the question of not only a possible common vascular mechanism is these disorders, but a common neural one as well. Loss of proprioception and deep pain sensation are essential to the genesis of Charcot joints and this has not been observed in primary resorptive arthropathy. As mentioned earlier, in addition to resorption, the typical Charot joint is distinguished by a marked effusion, instability, cartilage and bone destruction and fragmentation, bone sclerosis and hypertrophic changes, and the presence of intrasynovial necrotic bone and cartilage fragments, while radiographically as well as pathologically, only destructive and resorptive L_23~85,92-195 These classical neuropathic changes are seen in resorptive arthropathy. joint changes are usually seen in weight-bearing areas at some time in the course of a Charcot joint, but sclerosis and hypertrophic changes are often absent in neuropathic arthropathy involving the small bones of the wrists and the hands.‘96 Marked effusion in association with digital resorptive arthropathy has not been observed. Further, in Hansen’s disease and in syringomyelia where sensation is lost, resorption in the hand typically, but not invariably, proceeds
OPERA-GLASS
HAND
233
from the distal digital tuft proximally, rather than from the proximal interphalangeal or metacarpophalangeal joints. 64 In the foot, however, where pressure effects are maximal on the metatarsal heads rather than on the toe pads, the destruction in Hansen’s disease is first seen in the metatarsophalangeal joints and the pattern of bone and joint destruction closely resembles that of exception to this pattern of initial digital resorptive arthropathy. 9o Another tuft resorption in Hansen’s disease is seen when lepromatous granulomata cause primary invasive bone destruction.9’ The lack of overt clinical evidence of gross neuropathy in all reported cases and the absence of microscopic evidence of neural involvement in resorptive arthropathy both mitigate a major role for a neurological deficit in this syndrome. l-z3 Impaired sensation and a reduction of motor nerve conduction velocity in 43% of 70 cases of rheumatoid arthritis was reported by Good et al.19’ Despite this data, Chamberlain and Bruchner were unable to correlate the degree of joint involvement with motor or sensory conduction impairment in patients with rheumatoid arthritis. I98 Although complete examinations are not available on all of our cases, where sensory, motor nerve conduction, and/or electromyography was performed on six of our patients, the only abnormalities found were some decrease in size of evoked potentials in two cases and polyphasic potentials in the opponens and abductor digiti quinti in one patient.“’ Further, the recognized protective effect of hemiparesis and anterior poliomyelitis on the progression of rheumatoid arthritis argues against the role of sensory and motor impairment aggravating rheumatoid arthritis, although admittedly in these instances there is a joint protective factor of disuse.2W,20’ One additional factor of likely significance was the successful healing of a surgical fusion of a thumb interphalangeal joint in our patient, V.R. The failure of bone fusion is a characteristic of the neuropathic joint.ly4 Destructive
Synovitis
Because of the initial intra-articular focus of resorption, it is logical to presume a primary role for the intra-articular inflammatory reaction in the genesis of joint resorption. The invasive destructive feature of the rheumatoid granulation tissue, resulting in articular erosions and juxta-articular cyst formation Rubenstein, and DixonZo2 studied the has been long appreciated.18’ Jayson, pressure in large intraosseous juxta-articular cysts in two patients with rheumatoid arthritis and observed that increased intra-articular pressure associated with muscle contraction was transmitted directly to these cysts, suggesting that these cysts were extensions of the intra-articular destruction. Further, in a patient with a large cyst or “geode, ” in the distal radius, relaxation of the clenched fist often was not associated with a drop in intrageodal pressure. This suggested that the communication of the cyst to the joints became plugged, thereby permitting prolonged high intracystal pressure to persist, adding pressure necrosis to pannus invasion in the destruction of juxta-articular bone. Pressure Efects That intact of rheumatoid
bone can be eroded in rheumatoid arthritis by extrinsic pressure nodules has been amply demonstrated by Dorfman, Norman,
SWEZEY
ET AL.
Fig. 32. Case 22. Rheumatoid arthritis. Subluxation at the proximal interphalangeal joints has resulted in severe deformity of the fingers and pressure erosions of the adjacent articulating bones, which mimics the bone loss from resorption. Typical pencil and cup deformities of the metacarpophalangeal joints are present. Incidentally noted is endosteal cortical thickening of the distal phalanges which is occasionally seen in rheumatoid arthritis.
and Smith.3’ The supracondylar scalloping of the osteoporotic femur by the patella, the notching of the ribs in some arthritics, and the erosions caused by contiguous subluxed bones as seen in our Case 22 (Fig. 32) are other apparent examples of bone erosion by extrinsic forces. 30,203,204 Intraarticular pressure has been shown to increase with muscle tension and weight-bearing stress to levels well above normal hydrostatic pressure. 202,204,206 In the severely eroded rheumatoid joints, the protective cartilaginous cover is lost. This leaves exposed the osteoporotic subchondral bone which has poor resistance to compressive forces and is thus highly vulnerable to erosive and destructive actions.207m209 Pain and Joint A buse Following Bywater’s suggestion, Castillo, Sallab, and Scott studied the relationship of osteoporosis to bone cyst formation and found an inverse correlation.2’0,2” They also noted that cyst formation in rheumatoid arthritis was more prevalent in males and attributed the severe destructive cystic changes to continued abuse of diseased joints by patients with a high pain threshold.2’2 These findings contrast with the female predominance and marked bone demineralization of resorptive arthropathy, although it is interesting that Colton and Darby8,‘8.20,2’2 comment on the lack of pain associated with giant granulomatous lesions of the femoral head and neck in rheumatoid arthritis, a phenomenon alluded to by Blum, Mather, and Smyth8~‘8~20 and which we have also noted in resorptive arthropathy. Corticosteroid Eflects Although it is clear that resorptive arthropathy was recognized long before the advent of corticosteroid therapy, it is significant that apparent abuse of diseased joints rendered less painful by repeated intraarticular steroid has been associated with a severe destructive arthropathy.‘261’29,‘3’
B
W
44 F
F
F
52
59
56F
72 F
24F
W.S.
E.G.
V.B.
V.R.
V.L.
M.H.
?
47
?
?
43
19
31
5
?
33
23
37
21
17
2U
16
19
41
37
16
15
19
20
11
YEAFLSIWRATION AGE OF AGE OF ONSET OF ONSET ARI'HRITIS RJ3SORPTION ARTHRITIS
* JuvenileRheumatoidArthritis * -losing Spondylitis
W
B
W
W
W
40 F
A.B.
B
27F
D.T.
F’A!l?IR?l’AGE SMRACE
TABLE III
+
+
ThUlUbS
0
+
+
0
0
0
0
+
0
Wrists Elbows
0
PhalanX
R-OX.
Distal ulna Cerv. Spine
ThUmbS
Left Elbow
0
5
SlTES OF PSOFUASIS *JRAONSEl' +IcA.S. RESORPTIVEARTHRITIS HAND FEEZCUl'HER Wrists + + Elbows Shoulders Wrists + + Shoulders Elbow Hip Neck Knee
RESORPTIVEARTHROPA!l'BYANDTHEOPERA-GUSSHANDSYNDROME
-
-
+
+
+
E.C.
V.B.
V.R.
V.L.
Sjogren’s
-
-
+
-
-
-
RA NOW
1:2560
+ -
+
+
l-l-l
+
-
+
+
+
+
+
1:80
-
+
1~2560
Mild
Mild
-
LATEX ANA L.E. PRESENCEOF PAIN PRE!P m ARJZAOF FZUTION TITRE RESO~IVEARTRROPATHY
I-
syndrome
I -I-
-
W.S.
M.H.
+
D.T.
PATIEwr-s.s.
Raynaud'sphenomenon,auto-amputation of right fifth toe, PSS.
OTBEX co-
Normal
Noxmal
Normal
Normal
Normal
Successfulsurgicalfusion I.P. joint right thumb.
Rheumaticfever at age 14.
Raynaud's30 yrs. Sclerodexma diagnosis 12 yrs. ago.
Clinical SI.Zin 1951. Past history of treated syphilis.TPI+.
Normal prior Mid-cervicalatraumaticspinal cord It.4Cord corn-Icompression.
Normal
slw3ORYExAM
TABLE III (cont.)
OPERA-GLASS
237
HAND
OSTEOPOROSIS
Juxta-articular bone demineralization is a classical feature of rheumatoid arthritis. Soila demonstrated that this occurred even in the absence of actual pannus invasion.‘24 Recent investigations have demonstrated calcium releasing factors in rheumatoid synovial culture media.*14 Generalized bone demineralization unrelated to steroids was observed in 27.8:,;I of 36 patients studied by McConkey, Fraser, and Bligh.2’5 Duncan et al.*“examined rib biopsies in five patients with rheumatoid arthritis who were taking salicylates, but who had not taken steroid medication. All of these patients showed greater bone resorption than bone production. Thus, at least in the rheumatoid, not only is the juxta-articular bone subject to demineralization, but a generalized oseoporosis and susceptibility to trauma may exist. Synthesis
Of’Pathogenesis
We believe it reasonable to hypothesize the following mechanisms in an attempt to explain the pathogenesis of resorptive arthropathy. Initially, an aggressive inflammatory synovial lesion progressively destroys the cartilage and underlying bone. In the destructive process, terminal neural sensory filaments may also be destroyed (thereby creating a partial articular anesthesia similar to the consequences of a synovectomy). Ultimately, the entire synovium is destroyed and when this stage is reached, the painful inflammatory reaction can no longer progress. The exposed demineralized juxta-articular bone is thus subjected to intraarticular pressure stresses in excess of that which can be tolerated without causing pressure necrosis and resorption. Bone healing and remodeling are insufficient to restore normal contours and the typical result is tapered (“pencil”) and reciprocally hollowed (“cupped”) bone ends.‘s4 The initial inflammatory reaction, once having destroyed the joint, subsides, leaving in its wake only fibrous and fatty remnants for discovery by the pathologist at his belated arrival upon this bleak scene. ACKNOWLEDGMENTS Permission to reproduce Fig. 23 was kindly given by the editorial Medicine. I would like to thank of the patients described
Mark Epstein, in this report.
M.D.
for his generous
staff of the Archives
assistance
in making
of Internal available
two
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