Ankylosing spondylitis with childhood onset

April, 1969 T h e J o u r n a l of P E D I A T R I C S

Ankylosing

505

ondylitis with childhood onset

Seven male patients with childhood-onset ankylosing spondylitis are described. Ankylosing spondylitis should be considered in the differential diagnosis of childhood arthritis, especially if the patient is a male with onset of disease in late childhood, if there are any symptoms referable to the sacroiliac joints or lumbodorsal spine, or if there is transient peripheral arthritis affecting only a few joints. Initial symptoms are characteristically fleeting and recurrent. Early diagnosis and therapy are important for preservation of adequate function. The frequency o[ hip disease may be greater in individuals with early onset of disease.

Jane Schaller, M.D.,* Sam Bitnum, M.B., Ch.B., and Ralph J. Wedgwood, M.D. SEATTLE)

WASH.

ANKYLOSINO SPONDYLITIS is a type of chronic arthritis involving the sacroiliac joints with variable progression to joints of the lumbar, thoracic, and cervical spine. 1-~2 The disease is characterized by pain, stiffness, and loss of mobility of the back. Initial complaints are usually (80 per cent of patients) referable to the sacroiliac joints and low back and consist of pain and stiffness in the low back, buttocks, thighs, and hips; such symptoms are often transient and recurrent for years before obvious loss of spinal mobility occurs. In about 20 per cent of patients the disease begins with arthritis of peripheral joints, and in about one half of patients peripheral joints are affected at From the Department of Pediatrics, University of Washington, School of Medicine. Supported in part by Clinical Study Center Grant No. CRCS-36 /tom the Arthritis Foundation and the Pediatric Training Grant No. 5T1-AI-227 from the National Institutes o/ Health, United States Public Health Service. ~Address: Department ol Pediatrics, Un{verdty o[ Washington School of Medicine, Seattle, Wash. 98105.

some time during the course of disease. Peripheral arthritis is usually confined to a few large joints and is often transient and nondeforming. Iritis13, 14 and aortitis 15-~7 are two characteristic extra-articular manifestations; other systemic manifestations are not prominent. Diagnosis is based upon the clinical picture and the presence of radiographic changes of the sacroiliac joints; characteristic radiographic changes of the lumbodorsal spine appear later in the course of the disease. The relationship of ankylosing spondylitis to rheumatoid arthritis has been the subject of much controversy. Since both are diseases of uncertain cause, no conclusions concerning their etiologic relationships can be drawn. Histology of synoviaI tissue from affected joints is similar in both diseases. 1~ Ankylosing spondylitis clearly differs from rheumatoid arthritis in several respects, however: (1) characteristic involvement of sacroiliac joints and lumbodorsal spine with relative sparing of peripheral joints, (2) VoL 74, No. 4, pp. 505-516

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The Journal of Pediatrics April 1969

predilection for males (4 to 8 males to 1 female), (3) rarity of rheumatoid factor and rheumatoid nodules, (4) high fl'equency of iritis, 13, 14 (5) occurrence of a distinctive type of aortitis with aortic insufficiency,15-17 (6) therapeutic response to phenylbutazone and indomethacin, 19 but lack of response to gold, and (7) increased familial incidence of disease. Ankylosing spondylitis is usually considered a disease of young and middle-aged adult

males, and its occurrence before puberty has not been widely recognized. Although in retrospect about 10 per cent of patients in 3 large series~, 8, 11 had onset of symptoms before age 15 years, few cases of ankylosing spondylitis in childhood have been presented as such. The purpose of this report is to describe 7 patients with ankylosing spondylitis of childhood onset and to suggest that this disease should be considered in the differential diagnosis of childhood arthritis.

Case 1

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Fig. 2. Severe bilateral sacroiliac joint changes, Patient 1. (Onset of disease at age 7 years, radiograph at age 13 years.)

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CASE REPORTS Findings on all 7 patients are summarized in Table I. Descriptions of Patients 1 and 2 are presented in more detail. Patient 1 ( U H 138-58). This boy was well until age 7 years when he developed pain in the left hip, gluteal area, and thigh. He limped and had vague pain in the gluteal area on hip motion. Sedimentation rate was 21 ram. in 1 hour. Episodes of bilateral hip, thigh, and gluteal pain recurred. At age 10 years he complained of pain and tenderness of the lumbodorsal spine. Radiographs showed sclerosis and prominent osteolytic lesions of both sacroiliac joints; in retrospect, radiographs taken at age 7 showed early sacroiliac joint changes. Sedimentation rate was 61 mm. in 1 hour and hemoglobin, 10.1 Gin. Sacroiliac joint biopsy showed perichondritis with an infiltrate of lymphocytes and plasma ceils. Tuberculin test and brucella agglutinins were negative. Pain and swelling in both knees occurred at age 11 years, stiff neck at age 12, and arthritis of both wrists at age 13. Peripheral joint complaints were mild and of only 1 to 2 weeks' duration, but pain continued to recur in the hips and thighs. The patient remained active although vigorous exercise exacerbated his complaints. Rate of growth was noted to have slowed at age 7 years (Fig. 1). At age 13 years the patient was referred for evaluation of probable juvenile rheumatoid arth-

Fig. 3. Lumbar vertebral changes. (Patient 1, age I8 years.) Note anterior squaring of the body of L4.

Ankylosing spondylitis

507

rids. Significant physical findings included small stature, limited cervical spine mobility, and decreased motion of the left hip; there was no sacroiliac j o i n t tenderness or decreased lumbodorsal spine mobility. Sedimentation rate was 48 ram. in 1 hour and hematocrit, 37 per cent. Radiographs showed advanced erosions and sclerosis of both sacroiliac joints (Fig. 2). Symptomatic improvement occurred after treatment with salicylates and physical therapy. Between ages 13 and 18 years the patient had intermittent back pain with a progressive decrease in mobility of the entire spine. Intermittent hip pain and knee pain with swelling continued. A duodenal ulcer, which healed after antacid therapy, occurred at age 17 years. At age 18 years intermittent pain in both shoulders began. No cardiovascular, eye, or gastrointestinal symptoms had occurred. The patient had been only slightly limited in activity. Although pubertal changes began at age 13 years, rate of growth had remained slow. Physical examination at age 19 years showed that the patient had lost 75 per cent of lumbodorsal spine mobility and 25 per cent of cervicaI spine mobility. Chest expansion was 2 cm. Motion of both hips was limited. Peripheral joints appeared normal, as did the eyes and heart. Sedimentation rate was 40 mm. in 1 hour, hematocrit 38 per cent, latex agglutination and antinuclear factor negative, urinalysis normal, serum protein electrophoresis normal, and electrocardiogram normal. Radiographs showed further narrowing, sclerosis, and erosions of both sacroiliac joints, sclerosis and narrowing of the apophyseal joints from L a to the sacrum, and squaring of lumbar vertebral bodies (Fig. 3) with anterior ligamentous calcification. Films of cervical spine, hips, and peripheral joints were normal. There was no known familial occurrence of arthritis or spondylitis. Patient 2 ( U H 508-50), This boy was well until age 8 years when persistent pain in both groins and the left hip caused him to limp and decrease his physical activities. At age 9 ~ years an orthopedist found him to have low back tenderness and limited hip motion. Pelvic radiographs showed early changes in the right sacroiliac joint (Fig. 4, A). Sedimentation rate was 24 mm. in i hour, hemoglobin 11.3 Gm., and intradermal tuberculin test negative. When additional discomfort Began in both knees and the right foot, he was thought to have juvenile rheu-

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SchaIIer, Bitnum, and Wedgwood

The Journal o[ Pediatrics April 1969

C

Fig. 4. Progressive sacroiliac and hip changes, Patient 2. (Onset of disease at age 8 years.) A, Age 9 years. Early changes are present about the right sacroiliac joint. The left sacroiliac ioint and the hips appear normal; B, Age 1'5 years. Obvious bilateral sacroiliitis and bilateral hip changes are present; C, Age 16 years. Advanced destructive hip disease is now present. Both hip joint spaces have become narrowed and there are erosions of the femoral heads and acetabula.

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Ankylosing spondylitis

matoid arthritis and treated with salicylates. Intermittent episodes of pain in the hips, knees, and feet continued, however, and pain in the low back and mid-thoracic spine began. At age 11 years he was found to have marked lumbar lordosis, pain on motion of the hips, and painful, swollen knees. Sedimentation rate was 81 ram. in 1 hour. At age 1 1 ~ years he responded symptomatically to a short trial of phenylbutazone. In his twelfth year he experienced increasingly severe pain in his back and neck. Growth rate had slowed markedly since age 8 years (Fig. 5). At age 1 2 ~ years he was hospitalized. Physical examination showed a small boy with pain and stiffness of the entire spine who was unwilling to stand because of back, knee, and foot pain, and who could walk only with crutches. Chest expansion was 2 cm. Hip and jaw motion were limited; the knees, ankles, and right heel were swollen and painful. Sedimentation rate was 135 mm. in 1 hour. Radiographs showed erosions and sclerosis of both sacroiliac joints. The patient was treated with indomethacin, salicylates, and physical therapy. From ages 13 to 15~ years he improved remarkably in function although he lost nearly all lumbodorsal spine mobility. A duodenal ulcer discovered at age 13 years healed with antacid therapy. Sedimentation rates varied from 40 to 100 mm. in 1 hour and hematocrits from 28 to 32 per

cent. Antinuclear factor was transiently positive, and serum gamma globulin was elevated (2.1 Gm. per 100 ml.). At age 16 years multiple problems occurred: myocarditis, a 4 week episode of severe diarrhea, worsening of back pain, and severe arthritis affecting most peripheral joints including the small joints of the hands. The sedimentation rate rose to 126 mm. in 1 hour, and the hematocrit fell to 19 per cent. Steroid therapy was associated with rapid resolution of the myocarditis, slow improvement of joint symptoms, and reduction of sedimentation rate to normal. Recent physical examination (age 1 6 ~ years) showed a small boy in early puberty with mild cushingoid features, complete immobility of the lumbodorsal spine, tenderness over lumbar and sacroiliac joints, chest expansion of 1 cm., severe limitation of hip motion, waddling gait, normal peripheral joints, and occasional premature ventricular contractions. Ophthalmologic exam was normal. Radiographs showed advanced changes in both sacroiliac joints, squaring of vertebral bodies in the lumbodorsal spine, and bilateral destructive hip changes. Films of cervical spine and peripheral joints were normal (Figs. 4 and 6). The patient's father, now age 52 years, had full-blown ankylosing spondylitis which began at age 10 years with intermittent thigh pain and limp. He first noted back symptoms at age

Case 2

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5 10

Schaller, Bitnum, and Wedgwood

A

The Journal of Pediatrics April 1969

B

Fig. 6. Patient 2 at age 16 years, 3 months. A, Note growth retardation and maintenance

of reasonably good posture. B, Loss of lumbodorsaI spine mobility is obvious when the patient bends forward. 25 years and was found to have ankylosing spondylitis at age 27. He had had one attack of iritis (age 47 years) but no known cardiovascular or bowel disease. One of the father's brothers, age 59 years, had peripheral rheumatoid arthritis of 15 years' duration. DISCUSSION

General. Six of the 7 patients described here with childhood-onset ankylosing spondylitis had typical early manifestations of the disease. The onset occurred in late childhood in all patients (7 to 12 years of age). Three patients presented with low back, hip, groin, and thigh pain. Four patients presented with peripheral arthritis; knees were involved in 2 patients;'heels and metatarsals in l; and thumb, toes, and ankle in 1. Three of these patients developed symptoms referable to the sacroiliac joints and low back within 4 years of onset of peripheral joint symptoms. One patient had extensive peripheral joint disease and few symptoms referable to the back for 22 years before full-blown ankylosing spondylitis was recognized (Patient 5). In the 6 patients with early sacroiliac joint or low back complaints, these symptoms were

initially fleeting and recurrent and were either overlooked or misinterpreted. In all 6 of these patients the low back was affected symptomatically before the neck. Four patients had documented loss of mobility of the lumbodorsal spine before age 18 years, and 2 patients not examined early now have stiff spines in early adulthood. Six of 7 patients have decreased expansion of the chest; in 2 boys this was documented early in disease. Typical radiographic changes in the sacroiliac joints are now present in all patients (Figs. 2, 4, 7, A). In 3 patients studied early, changes were present within a few months of onset (Patient 1), within 1 year after onset (Patient 2), and 3 years after onset (Patient 4). In 2 patients initial changes were unilateral (Fig. 4). All but 1 patient (Patient 4) have radiographic changes of spondylitis in the lumbar spine (Fig. 3) ; 4 have changes in the thoracic spine, and 2 in the cervical spine (Fig. 7, B). Lumbar spine changes were documented by age 18 years in 3 patients who were examined early in the course of the disease. With the notable exception of hip disease,

Volume 74 Number 4

Ankylosing spondylitis

5 11

Fig. 7. Late radiographic changes of ankylosing spondyIitis. (Patient 6 at age 31 years.) A, Both sacroiliac joints are obliterated. Hip joint spaces are markedly narrowed. B, Apophyseal joints of the cervical spine are fused and there is anterior ligamentous calcification between several vertebral bodies (arrows). (This patient has a "bamboo spine" in the lumbar and thoracic areas.)

only 1 patient (Patient 5) has had destructive peripheral arthritis. In all others peripheral joint disease has been transient and nondeforming. Only 2 patients have had involvement of small joints of the hands. Peripheral joint disease has usually been asymmetric, involving only a few joints at a time. Affected peripheral joints have been warm, swollen and painful, appearing clinically as those i n rheumatoid arthritis. Three patients have complained of heel pain; only 1 (Patient 5) has calcaneal spurs on radiographs. Hip symptoms have occurred at some time in all patients. I t is difficult to define hip pain in ankylosing spondylitis since pain from sacroiliac joints can be poorly localized and referred to the hip region. However, all of these patients have had pain on hip motion or weight bearing; and all now have decreased hip motion on physical examination. Two patients (Patients 1 and 4) still have normal hip radiographs; the other 5 have radiographic evidence of femoral head destruction or narrowed hip joints. Three patients have had severe clinical hip disease (Patients 2, 5, 7) (Fig. 4). H i p symptoms began within 5 years of disease onset in 5 patients and at unknown times in 2. T h e fre-

quency of symptomatic and radiographic hip disease i n large series of patients with ankylosing spondylitis is about 25 per cent. 2, 7, 11, 12 Wilkinson and Bywaters 7 observed that hip and shoulder involvement occurred more frequently in patients with onset of disease before age 20 years. Severe hip disease is a major cause of disability in ankylosing spondylitis, as it is in late juvenile rheumatoid arthritis? ~ Systemic symptoms have not been prominent in these Patients , except for Patient 2 who has had severe anemia, probable early cardiac disease, and possible inflammatory bowel disease by age 16 years. None has yet developed iritis. The 2 patients with earliest onset have both had striking growth retardation dating from onset of their illness. Patient 2 (Figs. 5 and 6) remains in early puberty at age 1 6 ~ years. Patient 1 (Fig. 1) went through puberty without a growth spurt and remains of small stature at age 19 years; anabolic steroid therapy (oxandralone) from ages 1 5 ~ to 1 6 ~ years was associated with little increase in growth rate. In neither patient could corticosteroids or overt bowel disease be incriminated as possible causes of growth retardation. Growth retardation is

5 12

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Volume 74 Number 4

also known to occur in juvenile rheumatoid arthritis and other chronic inflammatory diseases of childrenY Five of 7 patients have had high sedimentation rates; in 4 patients levels greater than 40 mm. in 1 hour have persisted through years of folIow-up. None has had rheumatoid factor. Elevated serum gamma globulin and antinuclear factor were present in 1 patient (Patient 2). Few detailed descriptions are available of childhood-onset ankylosing spondylitis. Ten patients are described as such. 7' 22-25 The occurrence of ankylosing spondylitis in children under 15 years of age is also mentioned in other series, a-7, 11, 12, 1~, 2~-a0 With few exceptions, onset seems to be in children over 8 years of age. Initial symptoms and patterns of disease do not seem to differ from those recognized in adults, although more patients may present with peripheral arthritis. Ellefsen ~ noted severe hip disease in 2 of his 20 patients; other authors do not comment on frequency of severe hip disease or growth retardation. The occurrence of iritis reIatively early in disease is mentioned by Ellefsen 27 and Smiley2 l Smiley commented on the development of definite ankylosing spondylitis in 3 patients and possible ankylosing spondylitis in 4 patients who had previously been thought to have juvenile rheumatoid arthritis with iritis. No instances of early cardiac lesions are noted. Diagnosis. The diagnosis of ankylosing spondylitis cannot be made unless the disease is suspected, and herein lies the failure of recognition in most instances. The lag between onset and diagnosis of ankylosing spondylitis has been repeatedly commented on in the literature. Six of 7 children reported here had clear-cut symptoms of ankylosing spondylitis during childhood, yet only 2 were correctly diagnosed before age 18 years. Diagnosis depends upon: (1) symptoms of sacroiliac joint or low back pain, (2) demonstrable decreased mobility of the lumbar or lumbodorsal spine, and (3) radiographic sacroiliac changes. Aside from radiographs, there are no diagnostic laboratory

Ankylosing spondylitis

51 3

tests. T h e disease should be suspected in any child who has sacroiliac joint symptoms (low back, buttock, or thigh pain, or radiating pain into the legs) or back symptoms. It is important to realize that these symptoms may initially be intermittent and mild. Early physical findings may be minimal and include paravertebral muscle spasm and tenderness, and tenderness over the sacroiliac joints and lumbar spine. Pain on stress of the sacroiliac joints may be elicited by various orthopedic maneuversY, 82, 3a Flattening of the lumbar curve, most obvious when the patient bends forward, is usually the first sign referable to the back. Chest expansion may also be decreased, secondary to costovertebral involvement. Sacroiliac joint radiographic changes may be absent at onset but should appear within the first few years of disease. The disease should also be considered in any child with arthritis involving only a few peripheral joints, especially if the child is a boy with onset of disease past the age of 8 years and if the peripheral arthritis is transient and nondeforming. Such individuals should be watched for the occurrence of any complaints suggestive of sacroiliac joint, hip, or back involvement and should have radiographs of the sacroiliac joints. The most obvious differential diagnostic problem is juvenile rheumatoid arthritis. This disease bears many interesting similarities to ankylosing spondylitis: frequency of asymmetric peripheral arthritis involving the large joints; rarity of rheumatoid factor and rheumatoid nodules; frequency of iridocyclitis; and frequent involvement of sacroiliac joints and cervical spine. A family study of patients with juvenile rheumatoid arthritis showed an increased ;frequency of ankylosing spondylitis in relatives of children with rheumatoid arthritis; a4 however, this data may need reevaluation in view of possible development of ankylosing spondylitis by some of the probands. There are obviously a few patients who seem to have juvenile rheumatoid arthritis at onset but later develop typical ankylosing spondylitis. TM al There are also rare but interesting patients with severe destruc-

5 14

SchaUer, Bitnum, and Wedgwood

tive peripheral rheumatoid arthritis with onset in early childhood who later develop manifestations of ankylosing spondylitis with typical clinical and radiographic findings; 1~, a~, a6 the 2 syndromes seem to merge in such instances. In general, however, the diseases seem clinically distinct. Juvenile rheumatoid arthritis is associated with persistent peripheral arthritis. The spine involvement is cervical, not lumbodorsal. Although the sacroiliac joints are involved radiographically in about 25 per cent of patients, a7 low back symptoms are not prominent. Most patients with cervical spine arthritis and sacroiliac joint involvement of juvenile rheumatoid arthritis do not develop lumbodorsal involvement characteristic of ankylosing spondylitis, even after years of follow-up, s~ Sacroiliac joint radiographic involvement is generally less advanced than in anky!osing spondylitis and is often associated with hip disease, as The iridoeyclitis of juvenile rheumatoid arthritis is characteristically chronic and insidious, TM 39 as contrasted with the acute iritis associated with ankylosing spondylitis in adults. TM ~4 Other diseases associated with arthritis, such as acute rheumatic fever, systemic lupus erythematosus, dermatomyositis, scleroderma, and gout, should be readily separable on laboratory and clinical grounds 5tom ankylosing spondylitis. Other causes of hip girdle and back pain in children which should be considered in the differential diagnosis are infectious arthritis of the hips or sacroiliac joints (tuberculous or pyogenic), infection of intervertebral discs,4~ toxic synovitis of the hip, 4~ Legg-Perthes disease (aseptic necrosis of the femoral head), slipped capital femoral epiphysis, adolescent kyphosis (Scheuermann's disease), herniated intervertebral disc, spinal cord tumor, and vertebral fracture. Appropriate physical examination, radiographs, and laboratory tests will differentiate these conditions, as will careful follow-up of the clinical course. Three underlying conditions which have been associated with ankylosing spondylitis in adults should also be considered in children with spondylitis. These are psoriasis, 42 in-

The Journal of Pediatrics April 1969

flammatory bowel disease (ulcerative colitis~ regional enteritis), 4~45 and Reiter's sym drome. 46 Patient 2 in this series may have early bowel disease which was antedated symptomatically by 8 years of joint disease. Reiter's syndrome, associated with urethritis, conjunctivitis, and arthritis, has been described in children only rarely47; 1 patient with ankylosing spondylitis in Ellefsen's series 2~ had urethritis and conjunctivitis and may have represented an instance of Reiter's disease with spondylitis in childhood. Therapy. Therapeutic aims in ankylosing spondylitis are twofold: (1) relief of pain, (2) maintenance of good posture and function. Various antirheumatic drugs have been employed for relief of pain. Sali~.ylates, in antirheumatic dosage as used in rheumatoid arthritis, are sufficient in some patients and should be tried first in children since they are the safest available drugs. Several weeks or months may be needed for maximal response. Indomethacin 19 and phenylbutazone are useful drugs for relieving symptoms in ankylosing spondylitis, but must be used with care in children because of potential toxic side effects. Gold therapy is rarely helpful in ankylosing spondylitis in contrast to peripheral rheumatoid arthritis, and corticosteroids are rarely warranted in this chronic disease. Radiation therapy to the spine, which provides symptomatic relief in some patients, is not warranted in children because of possible induction of malignancy.48 Physical therapy is an extremely important part of treatment of ankylosing spondylitis. Patients need to be encouraged to maintain good posture. Appropriate exercises, well described by Blumberg and Ragan, ~ to promote posture and muscle strength should be prescribed and done regularly by the patient. Prolonged bed rest is to be avoided. The bed mattress should be firm (bed board) and large pillows avoided. Hot baths or showers may be effective in relieving pain and stiffness. Prognosis. The extent of spinal involvement which will occur in any given patient with ankylosing spondylitis is unpredictable. Usually much of the spine becomes involved

Volume 74 Number 4

over a period of years, although sometimes the disease may arrest without extensive spinal involvement. Even should fusion of the entire spine occur, over-all function will be adequate for usual activities if good posture has been maintained. About three fourths of patients with chronic ankylosing spondylitis remain employed and self-sufficient.5, 7 Significant peripheral arthritis, particularly hip disease, is potentially disabling. There are insufficient data to compare the prognosis of childhood-onset and adulthoodonset ankylosing spondylitis. T h e observations 7 that patients with ankylosing spondylitis of long duration have more spinal involvement and that patients with onset of spondylitis before age 20 may have more hip involvement might be interpreted to suggest a poorer ultimate prognosis for patients with early onset of disease. Four of the 7 present patients with childhood-onset spondylitis are currently doing well functionally, although 3 have already lost most spinal mobility. All have evidence of hip disease, however, and this may be a bad sign regarding future prognosis. Three of 7 patients have been significantly disabled, 1 by extensive peripheral arthritis (Patient 5), 1 by a combination of severe hip disease and social psychiatric problems (Patient 7), and 1 by severe back, hip, and systemic disease (Patient 9). The emotional aspects of chronic, painful illness such as ankylosing spondylitis in a child need emphasis. Promotion of the emotional and social development of the child is as important as curing the physical ill. Such children and their families need consistent, sympathetic encouragement. Children should be encouraged to maintain activities as normal as possible and should not be allowed to develop the seIf-image of chronic invalidism. Children with handicaps will need help in planning for a realistic future and vocation. SUMMARY

Seven patients with childhood-onset ankylosing spondylitis are described. All were males with onset of disease between 7 and 12 years of age. Three had symptoms referable

Ankylosing spondylitis

5 15

to the sacroiliac joints and low back at onset. Four presented with peripheral arthritis; 3 of these patients developed sacroiliac joint and back symptoms within 4 years of onset. Six of 7 patients have now lost most lumbodorsal spine motion. All 7 patients have had symptomatic hip disease, 5 with abnormal hip radiographs and 3 with severe clinical hip disease. All patients have had peripheral joint manifestations at some time; in 6 patients peripheral arthritis (excluding hip involvement) has been transient and nondeforming. Four patients currently have adequate function for nearly full activities, 1 is moderately disabled, and 2 are severely disabled. Marked growth retardation has been present in the 2 patients with earliest disease onset. One patient may have early cardiovascular manifestations and inflammatory bowel disease. None has yet had iritis. Ankylosing spondylitis should be considered in the differential diagnosis of childhood arthritis. Initial symptoms may be transient and recurrent. Early diagnosis and therapy are important for preservation of adequate function. The authors thank Drs. Vincent Kelley and George Limbeck for referring Patient 1, Dr. Ernest Burgess for referring Patient 2, and Dr. Louis Fey for referring Patient 6. We are indebted to Dr. Benjamin Graham for his assistance in reviewing the radiographs. REFERENCES

1. Boland, E, W., and Present, A. J.: Rheumatoid spondylitis: A study of 100 cases with special reference to diagnostic criteria, J. A. M. A. 129: 843, 1945. 2. Polley, H. F., and Sloeumb, C. H.: Rheumatoid spondylitis: A study of 1035 cases, Ann. Int. Med. 26: 240, 1947. 3. Foresterier, J., Jacqueline, F., and RotesQuerol, J.: Ankylosing spondylitis: Clinical considerations, roentgenology, pathologic anatomy, treatment, Translated by A. U. Desjardins, Springfield, Ill., 1956, Charles C Thomas, Publisher. 4. Romanus, R.: Pelvo-spondylitls ossificans in the male and genitourinary infection, Acta med. scandinav, suppl. 280: 1, 1953. 5. Blumberg, B., and Ragan, C.: The natural history of rheumatoid spondylitis, Medicine 35- 1, 1956. 6. Hart, F. D., and Maclagan, N. F.: Ankylos-

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7. 8. 9. 10. 11.

12.

13. 14. 15.

16. 17. 18. 19. 20.

21. 22. 23. 24. 25. 26. 27. 28.

ing spondylitis. A review of 184 cases, Ann. Rheum. Dis. 14: 77, 1955. Wilkinson, M., and Bywaters, E. G. L.: Clinical features and course of ankylosing spondylitis, Ann. Rheum. Dis. 17: 209, 1958. Lefkovits, A. M., and Thomas, J. R.: Rheumatoid spondylitis: Manifestations and management, Ann. Int. Med. 49: 89, 1958. Graham, D. C.: Ankylosing spondylitis, Canad. M. A. J. 82: 671, 1960. Mikkelsen, W. M., and Duff, I. F.: Rheumatoid (ankylosing) spondylitis, M. Clin. North America 45: 1307, 1961. Julkunun, H.: Rheumatoid spondylitis: Clinical and laboratory study of 149 cases compared with 182 cases of rheumatoid arthritis, Acta rheum, scandinav, suppl. 4: 1, 1962. Dilsen, N., McEwen, C., Poppel, M., Gersh, W. J., DiTata, D., and Carmel, P.: A comparative roentgenologieal study of rheumatoid arthritis and rheumatoid (ankylosing) spondylitis, Arth. & Rheum. 5: 341, 1962. tIaarr, M.: Rheumatic iridocyclitis, Acta ophth. 38: 37, 1960. Stanworth, A., and Sharp, J.: Uveitis and rheumatic diseases, Ann. Rheum. Dis. 15: 140, 1956. Schilder, D. P., Harvey, W. P., and Hufnagel, C. A.: Rheumatoid spondylitis and aortic insufficiency, New England J. Med. 255: 11, 1956. Clark, W. S., Kulka, J. P., and Bauer, W.: Rheumatoid aortitis with aortic regurgitation, Am. J. Med. 22: 580, 1957. Graham, D. C., and Smythe, H. A.: The carditis and aortitis of ankylosing spondylitis, Bull. Rheum. Dis. 9: 171, 1958. Cruickshank, B.: Pathology of ankyloslng spondylitis, Bull. Rheum. Dis. 10: 211, 1960. Calabro, J. J., and Amante, C. M.: Indomethacin in ankylosing spondylitis, Arth. & Rheum. 11: 56, 1968. Jeremy, R., Schaller, J., Arkless, R. A., Wedgwood, R. J., and Healey, L. A.: Juvenile rheumatoid arthritis persisting into adulthood, Am. J. Med. 45: 419, 1968. Ansell, B. M., and Bywaters, E. G. L.: Growth in Still's disease, Ann. Rheum. Dis. 15: 295, 1956. Lynn, T. N.: Rheumatoid spondylitis in a prepubertal female, Am. J. Dis. Child. 91: 158, 1956. Edstr6m, .G., Thune, S., and WittbomCig~n, G.: Juvenile ankylosing spondylitis, Acta rheum, scandinav. 6: 161, 1960. AnsellI B. M., and Bywaters, E. G. L.: Diagnosis of "probable" Still's disease and its outcome, Ann. Rheum. Dis. 21: 253, 1962. Jacobs, P.: Ankylosing spondylitis in children and adolescents, Arch. Dis. Childhood 38: 492, 1963. Adler, E., and Carmon, A.: Ankylosing spondylitis--a review of 115 cases, Acta rheum, scandinav. 7: 219, 1961. Ellefsen, F.: Juvenile ankylosing spondylitis, Acta rheum, scandinav. 13: 14, 1967. Simpson, N. R. W., and Stevenson, C. J.:

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