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Rehabilitative management of polyarteritis: A case report
89
CLINICAL
NOTES
Rehabilitative Jon Mukand,
Management
of Polyarteritis:
A Case Report
MD, PhD, Leo Langlois, MD, William Sniger, MD
ABSTRACT. Mukand J, Langlois L, Sniger W. Rehabilitative management of polyarteritis: a case report. Arch Phys Med Rehabil 1997;78:89-91.
OLYARTERITIS (PA) was first described in 1866 by Kussmaul and Maier, who noted the palpable arterial nodules that appear late in the course of this form of systemic necrotizing angiitis. PA is the prototype of the systemic necrotizing vasculitides and primarily affects small and medium arteries. Polyarteritis may involve any organ, but the classic form affects the kidneys, heart, gastrointestinal tract, skin, joints, and peripheral nervous system. The pulmonary vasculature is spared, in contrast to its less common variant, Churg-Strauss disease, in which asthma and eosinophilia are present. These clinical manifestations distinguish it from the other vasculitic syndromes. For instance, Kawasaki disease is an acute infantile febrile mucocutaneous syndrome that causes coronary arteritis; Wegener’s granulomatosis involves a chronic progressive inflammation of the respiratory tract and glomerulonephritis; Henoch-Schonlein vasculitis causes purpura, abdominal complaints, arthralgia and glomerulonephritis; giant cell arteritis affects cranial arteries and the aorta, causing headaches, visual loss, and the aortic arch syndrome; Takayasu arteritis causes arterial occlusion, leading to claudication, hypertension, absent pulses, angina, and aortic insufficiency, in addition to skin lesions that are similar to erythema nodosum. Polyarteritis is an uncommon disease, with an estimated annual incidence rate of 0.7 per 100,000 population and a prevalence rate of 6.3 per 100,000 in the United States.’ It occurs more commonly in males, with a sex ratio of 2-3:l. All ages
may be affected, but the peak incidence ranges from 40 to 60 years and it is observed in all racial groups.3 There have been several studies reporting an association of PA and recent hepatitis B virus infection,4 although the prevalence of hepatitis B surface antigenemia varies from 6% to 30%.3 A PA-like vasculitis with associated peripheral neuropathy has been reported in patients with human immunodeficiency virus.’ Other associated viruses may include cytomegalovirus, hepatitis A, human Tcell leukemia virus type I, and parvovirus.’ The exact cause of the arteritis is not known, but the most commonly considered mechanism is the deposition of circulating immune complexes. As in serum sickness, antigen excess leads to soluble antigen-antibody complexes that are deposited in blood vessel walls. The IgG and IgM immunoglobulins activate complement components such as C5a, a powerful chemotactic agent for neutrophils, which infiltrate the arterial wall and release enzymes responsible for vascular injury. The vasculitis associated with hepatitis B antigenemia is often cited to support this theory, with hepatitis B surface antigen having been demonstrated in the circulating immune complexes and involved tissues.4 The prognosis is poor when PA is left untreated but improves with corticosteroid therapy.3 Whereas only 13% of untreated patients may survive 5 years, with steroid treatment the survival rate ranges from 48% to 63%.‘j The role of cytotoxic drug therapy, however, continues to be debated. A recent prospective randomized study found that the addition of cyclophosphamide to a regimen of prednisone and plasmapheresis did not significantly affect the lo-year cumulative survival rates for patients with PA.’ The prognosis of this disorder depends on the severity and reversibility of the initial visceral involvement, as well as the treatment approach. Poor prognostic factors include impaired renal function, cardiac failure, gastrointestinal involvement, and age greater than 50 years.6,7 We report a case of a woman who developed quadriplegia and severe distal peripheral neuropathy secondary to polyarteritis. With timely and appropriate medical care, followed by an intensive rehabilitation program, she made significant progress. Although a case of microscopic polyarteritis nodosa accompanied by subacute peripheral neuropathy has been reported,8 a review of recent literature failed to locate any articles about the rehabilitation of patients with neuropathy due to PA. This case report discusses the rehabilitative management of this uncommon disease.
From the Southern NE Rehabilitation Center, Providence, RI, the Department of Rehabilitation Medicine. Boston Universitv. and the Deuartment of Orthooedics and Rehabilitation Medicine, Brown University (Dr. Mukand): the Department of Rehabilitation Medicine, Walter Reed Army Medical Center, Bethesda, MD (Dr. Langlois); and the Department of Rehabilitation Medicine, Boston University Medical Center (Dr. Sniger). Submitted for publication December 15, 1995. Accepted in revised form April 3, 1996. No commercial party having a direct financial interest in the results of the research supporting this article has or will confer a benefit upon the authors or upon any organization with which the authors are associated. Reprint requests to Jon A. Mukand, MD, PhD, Department of Rehabilitation Medicine, Southern New England Rehabilitation Center. 21 Peace Street, Providence, RI 02907. 0 1997 by the American Congress of Rehabilitation Medicine and the American Academy of Physical Medicine and Rehabilitation 0003.9993/97/7801-3813$3.00/O
The patient was a 75-year-old woman who was in good health, with complete functional independence, until 2 weeks before admission. She began to experience bilateral lower limb “heaviness,” progressing to pain and weakness below the knees and resulting in decreased mobility. In 1 week, she became unable to ambulate and complained of numbness and swelling in all four extremities. Her upper limbs were weaker than her lower extremities, and worse distally than proximally. Her medical history was significant for a right knee total arthroplasty, performed five years previously to treat severe osteoarthritis. The patient was admitted with a diagnosis of right lower extremity cellulitis and progressive muscle weakness. After
A 75year-old woman with polyarteritis who developed polyneuropathy and quadriplegia underwent intensive rehabilitation that resulted in significant improvement. This report discusses various therapeutic strategies for the successful management of patients with severe polyarteritis. Strategies include orthotics for both upper and lower extremities, sensory reeducation, edema management, and the use of adaptive devices in retraining the patient with activities of daily living. The associated neurological, orthopedic, renal, and cardiac complications in the context of rehabilitation for this complex condition are discussed. 0 1997 by the American Congress ofRehabilitation Medicine and the American Academy of Physical Medicine and Rehabilitation
P
CASE
Arch
REPORT
Phys Med
Rehabil
Vol78,
January
1997
90
REHABILITATION
FOR POLYARTERITIS,
evaluation, it was determined that she had a diffuse distal peripheral neuropathy and quadriplegia secondary to polyarteritis. Electrodiagnostic studies showed signs of acute denervation and axonal neuropathy suggestive of mononeuritis multiplex. Peripheral nerve and lymph node biopsies confirmed the diagnosis of vasculitis secondary to polyarteritis. Cerebrospinal fluid culture findings were negative, and chest x-ray, electrocardiogram, and echocardiogram findings were within normal limits. Computed tomography of the neck showed minimal asymmetric enlargement of the right masseter and left lobe of the thyroid, which was of no clinical significance. Moderate leukocytosis was noted on admission, but soon decreased to within normal limits. She was treated and monitored on a regimen of prednisone and Cytoxan for 1 month while on the acute medical service. Prednisone was continued during her rehabilitation stay, but Cytoxan was withheld for the first 3 weeks because of a low white cell count (2.6 k/bL). Her hospitalization was complicated by recurrent urinary tract infections and an episode of depression, which was treated with Triavil. On admission to the rehabilitation service, the patient presented as a pleasant, alert, and oriented, mildly obese, elderly woman. She was capable of a nearly perfect score on the Folstein mini-mental status exam, but exhibited slightly flattened affect. The examination found profound distal muscle weakness of all extremities, with only trace movement in her hands, feet, and ankles. She had 3-415 strength in both shoulders and the left elbow, and only l/5 in the left wrist. She had 2-31 5 right elbow strength, and no muscle function in the right wrist. Her lower limbs had grades of 3-4/5 bilaterally in the hips and knees, decreasing distally to absent function in the ankle and foot. Sensory function was diminished in a proximal-to-distal pattern. There was complete anesthesia distal to the ankles and wrists bilaterally, but sensation was intact proximal to both knees, the left elbow, and the right humerus at the midshaft level. Muscle stretch reflexes were not obtainable in any limb and no plantar responses were elicited. Cerebellar examination was unremarkable for problems with balance or coordination, except those related to muscle weakness. She was dependent for all activities of daily living (ADL). She required maximum assistance for transfers, was nonambulatory, and was dependent on wheelchair for mobility. The patient was started on an intensive program of inpatient rehabilitation lasting more than 2 months. She engaged in therapeutic exercises and received transfer training and wheelchair mobility training. Functional electrical stimulation (FES) was also used to improve muscle recovery in her upper and lower extremities, but a trial suggested little potential for benefit and the treatment was discontinued. She was fitted with bilateral metal, double upright ankle-foot orthoses (AFOs) and received gait training using a platform walker. The occupational therapist fitted her with bilateral wrist splints that were custom-molded and padded. She learned the use of appropriate adaptive equipment for ADL training. For sensory reeducation, the occupational therapist used a variety of materials including rice, shaving cream, gel, and powder. The patient required Isotoner gloves, Ace wraps, and elastic stockings to control the edema in her extremities. Neuropsychology personnel evaluated and monitored the patient’s cognitive status and mood for signs of recurrent depression. To prevent contractures, the nursing staff applied bilateral lower extremity boots when the patient was in bed and also monitored her skin integrity. One week after admission, the patient developed questionable electrocardiographic changes and was transferred to the acute setting for three days; when no evidence of a cardiac event was found, she was returned to the rehabilitation center. Three weeks
Arch
Phys Med
Rehabil
Vol78,
January
1997
Mukand
later, she had increased right ankle edema and ecchymosis, but denied having pain or a history of trauma. X-ray showed a small avulsion fracture of the tip of the right lateral malleolus and a healing fracture of the right fifth proximal phalanx. Because of her profound sensory deficits in the distal lower extremities, the most likely etiology of these fractures was trauma during transfers or ambulation. She was restricted to non-weight-bearing status for 1 week and then returned to full weight-bearing with her bilateral AFOs without experiencing problems. She also required a urology consultation for a bloody urethral discharge and a history of recurrent urinary tract infections. She was diagnosed with urethral prolapse and corrective surgery was planned. The patient progressed steadily throughout her rehabilitative course despite these minor setbacks. Upper limb strength improved approximately one muscle grade from admission and there was some functional return of the left wrist and hand for gross motor tasks. Her sensory function remained severely compromised, but with adaptive equipment she could feed herself and required only minimal assistance for dressing and bathing. Her transfer ability improved from requiring maximum assistance at admission to needing only supervision. She was completely independent in bed mobility. She could ambulate safely with her AFOs and a platform walker for 100 feet with only contact guard assistance. Her overall FIM score rose from 61 at admission to 81 at discharge. She still required maximal assistance for bathing and for dressing the lower extremities. Toileting and toilet transfers were with supervision. Because of several factors, she was unable to return to her home. Although her family members were supportive, they were unable to provide supervision and there were concerns for her safety. In addition, her home would have required major architectural modifications to become wheelchair-accessible. Therefore, she was transferred to a skilled nursing facility for additional rehabilitation.
DISCUSSION This case illustrates the various issues involved in the rehabilitative management of a patient with polyarteritis, including some of the important complications associated with this condition. Peripheral nerves may be involved in 50% to 70% of patients with polyarteritis and, as in this case, may lead to the initial presentation.2,6.9 Arteritis of the vasa nervorum located in the epineurium results in widespread nerve fiber degeneration. Unlike diabetic peripheral neuropathy, the upper and lower extremities are equally likely to be affected. Pain and paresthesias in a particular peripheral nerve distribution precede a motor deficit. A multiple mononeuropathy ensues, often progressing to a symmetrical polyneuropathy that involves motor function and every sensory modality. As with any peripheral neuropathy, the principles of skin integrity, hygiene, and joint protection are of paramount importance. Effective patient education and adequate control of dependent edema to prevent skin breakdown are especially important, because acute and often permanent sensory losses occur. Stress fractures of the feet may or may not be avoidable, and should be suspected if there is new onset of edema or ecchymosis because pain may not be a symptom. Proper orthotic devices may reduce the incidence of this complication. Metal and leather AFOs are preferable for optimal support, skin integrity, and durability if the patient has the potential to be ambulatory. The extra weight may also help increase proprioceptive feedback to the patient’s less involved proximal joints and thus improve gait training. With upper limb involvement, wrist splints are an essential
REHABILITATION
FOR POLYARTERITIS,
adjunct to occupational therapy for protecting anesthetic extremities and improving proprioception. In addition, forearm troughs attached to a front-wheeled walker can result in profound improvement in ambulation. They allow more weightbearing as the strong proximal shoulder depressors can compensate for the distal motor deficits. As a result, patients gain more confidence and control of ambulation. The heart is affected in up to 75% of cases with PA studied at autopsy,” and the findings include cardiac hypertrophy, pericarditis, coronary arteritis, and myocardial infarction.” Notably, cardiac involvement is often clinically silent, and one study of 165 patients found only an 18% incidence of cardiac failure.6 Despite this, heart failure contributes to death in up to 50% of the cases; signs of cardiac compromise must be investigated, as was done when our patient had some questionable electrocardiographic changes. Patients should be carefully monitored for cardiac complications. Renal involvement results in nearly 65% of the deaths associated with classic PA.‘,” Autopsy studies indicate that up to 85% of cases may be affected with either renal polyarteritis (60%) or glomerulonephritis (25%).“.r3 The clinical manifestation of proteinuria is common and occasionally a nephrotic syndrome develops. Microscopic and even macroscopic hematuria may occur with progressive renal failure due to glomerulonephritis. Secondary renal hypertension is present in about 30% of the cases, but is usually amenable to pharmacologic therapy.6 Our patient did not have any serious renal complications; her bloody urethral discharge was unrelated to the arteritis. A thorough evaluation of genitourinary complaints in this patient population is always warranted, given the potential for fatal renal complications.
working with these patients, one must remain vigilant for the serious cardiac and renal complications of this disease and be prepared to make appropriate management decisions when necessary. I.
2. 3. 4. 5. 6. I.
8. 9. 10.
CONCLUSION Polyarteritis is an uncommon disease with the potential for multiple systemic complications. Peripheral neuropathy with associated motor weakness affects more than half of the patients, making them candidates for rehabilitative services. Unlike other polyneuropathies, such as Guillain-Barre syndrome, neuropathy related to PA has a much poorer prognosis, but this does not preclude successful rehabilitative outcomes. When
91
Mukand
11. 12. 13.
References Kussmaul A, Maier R. Ueber eine bisher nicht beshriebene eigenthumliche arterienerkrankung die mit Morbus Brightii und rapid fortschreitender allgemeiner Muskellahmung einhergeht. Dtsch Arch Klin Med 1866; 1:483-51X. Conn DL. Polyarteritis. Rheum Dis Clin North Am 1990; 16:34162. Michet CJ. Epidemiology of vasculitis. Rheum Dis Clin North Am 1990; 16:261+X. Gocke DJ, Morgan C, Lockshin M, Hsu K, Bombardieri S, Christian CL. Association between polyarteritis and Australia antigen. Lancet 1970;2:1149-53. Libman BS, Quismorio FP Jr, Stimmier MM. Polyarteritis nodosalike vasculitis in human immunodeficiency virus infection. J Rheumatol 1995;22:351-5. Guillevin L, Du LTH, Godeau P, Jais P, Wechslet B. Clinical findings and prognosis of polyarteritis nodosa and Churg-Strauss angiitis: a study in 165 patients. Br .I Rheumatol 1988;27:258-64. Guillevin L, Jarrousse B, Lok C, Lhote F, Jais JP: Le Thi Huong Du D, et al. Longterm follow-up after treatment of polyarteritis nodosa and Churg-Strauss angiitis with comparison of steroids, plasma exchange and cyclophosphamide to steroids and plasma exchange: a prospective randomized trial of 71 patients. J Rheumato1 1991; 18:567-74. Oguni E, Iwamoto H, Nagata H, Yamaguchi N, Mizusawa H. A case of microscopic polyarteritis nodosa accompanied by peripheral neuropathy. Rinsho Shinkeigaku 1994;34:497-9. Frohnert PP, Sheps SG. Long-term follow-up study of periarteiitis nodosa. Am J Med 1967;43:8-14. Robbins SL, Cotran RS, Kumar V. Inflammatory diseases-the vasculitides. In: Robbins SL, Cotran RS: Kumar V, editors. Robbins pathologic basis of disease. 4th ed. Philadelphia: Saunders, 1989: 570-3. Holsinger DR; Osmundson PJ, Edwards JE. The heart in periarteritis nodosa. Circulation 1962;25:610-8. Rose GA. The natural history of periarteritis. BMJ 1957;2: 1148. 52. Wolff SM. Polyarteritis nodosa group. In: Wyngaarden JB, Smith LH, Bennett JC, editors. Cecil’s textbook of medicine. 19th ed. Philadelphia: Saunders, 1992: 1539-41.
Arch
Phys Med
Rehabil
Vol78,
January
1997
CLINICAL
NOTES
Rehabilitative Jon Mukand,
Management
of Polyarteritis:
A Case Report
MD, PhD, Leo Langlois, MD, William Sniger, MD
ABSTRACT. Mukand J, Langlois L, Sniger W. Rehabilitative management of polyarteritis: a case report. Arch Phys Med Rehabil 1997;78:89-91.
OLYARTERITIS (PA) was first described in 1866 by Kussmaul and Maier, who noted the palpable arterial nodules that appear late in the course of this form of systemic necrotizing angiitis. PA is the prototype of the systemic necrotizing vasculitides and primarily affects small and medium arteries. Polyarteritis may involve any organ, but the classic form affects the kidneys, heart, gastrointestinal tract, skin, joints, and peripheral nervous system. The pulmonary vasculature is spared, in contrast to its less common variant, Churg-Strauss disease, in which asthma and eosinophilia are present. These clinical manifestations distinguish it from the other vasculitic syndromes. For instance, Kawasaki disease is an acute infantile febrile mucocutaneous syndrome that causes coronary arteritis; Wegener’s granulomatosis involves a chronic progressive inflammation of the respiratory tract and glomerulonephritis; Henoch-Schonlein vasculitis causes purpura, abdominal complaints, arthralgia and glomerulonephritis; giant cell arteritis affects cranial arteries and the aorta, causing headaches, visual loss, and the aortic arch syndrome; Takayasu arteritis causes arterial occlusion, leading to claudication, hypertension, absent pulses, angina, and aortic insufficiency, in addition to skin lesions that are similar to erythema nodosum. Polyarteritis is an uncommon disease, with an estimated annual incidence rate of 0.7 per 100,000 population and a prevalence rate of 6.3 per 100,000 in the United States.’ It occurs more commonly in males, with a sex ratio of 2-3:l. All ages
may be affected, but the peak incidence ranges from 40 to 60 years and it is observed in all racial groups.3 There have been several studies reporting an association of PA and recent hepatitis B virus infection,4 although the prevalence of hepatitis B surface antigenemia varies from 6% to 30%.3 A PA-like vasculitis with associated peripheral neuropathy has been reported in patients with human immunodeficiency virus.’ Other associated viruses may include cytomegalovirus, hepatitis A, human Tcell leukemia virus type I, and parvovirus.’ The exact cause of the arteritis is not known, but the most commonly considered mechanism is the deposition of circulating immune complexes. As in serum sickness, antigen excess leads to soluble antigen-antibody complexes that are deposited in blood vessel walls. The IgG and IgM immunoglobulins activate complement components such as C5a, a powerful chemotactic agent for neutrophils, which infiltrate the arterial wall and release enzymes responsible for vascular injury. The vasculitis associated with hepatitis B antigenemia is often cited to support this theory, with hepatitis B surface antigen having been demonstrated in the circulating immune complexes and involved tissues.4 The prognosis is poor when PA is left untreated but improves with corticosteroid therapy.3 Whereas only 13% of untreated patients may survive 5 years, with steroid treatment the survival rate ranges from 48% to 63%.‘j The role of cytotoxic drug therapy, however, continues to be debated. A recent prospective randomized study found that the addition of cyclophosphamide to a regimen of prednisone and plasmapheresis did not significantly affect the lo-year cumulative survival rates for patients with PA.’ The prognosis of this disorder depends on the severity and reversibility of the initial visceral involvement, as well as the treatment approach. Poor prognostic factors include impaired renal function, cardiac failure, gastrointestinal involvement, and age greater than 50 years.6,7 We report a case of a woman who developed quadriplegia and severe distal peripheral neuropathy secondary to polyarteritis. With timely and appropriate medical care, followed by an intensive rehabilitation program, she made significant progress. Although a case of microscopic polyarteritis nodosa accompanied by subacute peripheral neuropathy has been reported,8 a review of recent literature failed to locate any articles about the rehabilitation of patients with neuropathy due to PA. This case report discusses the rehabilitative management of this uncommon disease.
From the Southern NE Rehabilitation Center, Providence, RI, the Department of Rehabilitation Medicine. Boston Universitv. and the Deuartment of Orthooedics and Rehabilitation Medicine, Brown University (Dr. Mukand): the Department of Rehabilitation Medicine, Walter Reed Army Medical Center, Bethesda, MD (Dr. Langlois); and the Department of Rehabilitation Medicine, Boston University Medical Center (Dr. Sniger). Submitted for publication December 15, 1995. Accepted in revised form April 3, 1996. No commercial party having a direct financial interest in the results of the research supporting this article has or will confer a benefit upon the authors or upon any organization with which the authors are associated. Reprint requests to Jon A. Mukand, MD, PhD, Department of Rehabilitation Medicine, Southern New England Rehabilitation Center. 21 Peace Street, Providence, RI 02907. 0 1997 by the American Congress of Rehabilitation Medicine and the American Academy of Physical Medicine and Rehabilitation 0003.9993/97/7801-3813$3.00/O
The patient was a 75-year-old woman who was in good health, with complete functional independence, until 2 weeks before admission. She began to experience bilateral lower limb “heaviness,” progressing to pain and weakness below the knees and resulting in decreased mobility. In 1 week, she became unable to ambulate and complained of numbness and swelling in all four extremities. Her upper limbs were weaker than her lower extremities, and worse distally than proximally. Her medical history was significant for a right knee total arthroplasty, performed five years previously to treat severe osteoarthritis. The patient was admitted with a diagnosis of right lower extremity cellulitis and progressive muscle weakness. After
A 75year-old woman with polyarteritis who developed polyneuropathy and quadriplegia underwent intensive rehabilitation that resulted in significant improvement. This report discusses various therapeutic strategies for the successful management of patients with severe polyarteritis. Strategies include orthotics for both upper and lower extremities, sensory reeducation, edema management, and the use of adaptive devices in retraining the patient with activities of daily living. The associated neurological, orthopedic, renal, and cardiac complications in the context of rehabilitation for this complex condition are discussed. 0 1997 by the American Congress ofRehabilitation Medicine and the American Academy of Physical Medicine and Rehabilitation
P
CASE
Arch
REPORT
Phys Med
Rehabil
Vol78,
January
1997
90
REHABILITATION
FOR POLYARTERITIS,
evaluation, it was determined that she had a diffuse distal peripheral neuropathy and quadriplegia secondary to polyarteritis. Electrodiagnostic studies showed signs of acute denervation and axonal neuropathy suggestive of mononeuritis multiplex. Peripheral nerve and lymph node biopsies confirmed the diagnosis of vasculitis secondary to polyarteritis. Cerebrospinal fluid culture findings were negative, and chest x-ray, electrocardiogram, and echocardiogram findings were within normal limits. Computed tomography of the neck showed minimal asymmetric enlargement of the right masseter and left lobe of the thyroid, which was of no clinical significance. Moderate leukocytosis was noted on admission, but soon decreased to within normal limits. She was treated and monitored on a regimen of prednisone and Cytoxan for 1 month while on the acute medical service. Prednisone was continued during her rehabilitation stay, but Cytoxan was withheld for the first 3 weeks because of a low white cell count (2.6 k/bL). Her hospitalization was complicated by recurrent urinary tract infections and an episode of depression, which was treated with Triavil. On admission to the rehabilitation service, the patient presented as a pleasant, alert, and oriented, mildly obese, elderly woman. She was capable of a nearly perfect score on the Folstein mini-mental status exam, but exhibited slightly flattened affect. The examination found profound distal muscle weakness of all extremities, with only trace movement in her hands, feet, and ankles. She had 3-415 strength in both shoulders and the left elbow, and only l/5 in the left wrist. She had 2-31 5 right elbow strength, and no muscle function in the right wrist. Her lower limbs had grades of 3-4/5 bilaterally in the hips and knees, decreasing distally to absent function in the ankle and foot. Sensory function was diminished in a proximal-to-distal pattern. There was complete anesthesia distal to the ankles and wrists bilaterally, but sensation was intact proximal to both knees, the left elbow, and the right humerus at the midshaft level. Muscle stretch reflexes were not obtainable in any limb and no plantar responses were elicited. Cerebellar examination was unremarkable for problems with balance or coordination, except those related to muscle weakness. She was dependent for all activities of daily living (ADL). She required maximum assistance for transfers, was nonambulatory, and was dependent on wheelchair for mobility. The patient was started on an intensive program of inpatient rehabilitation lasting more than 2 months. She engaged in therapeutic exercises and received transfer training and wheelchair mobility training. Functional electrical stimulation (FES) was also used to improve muscle recovery in her upper and lower extremities, but a trial suggested little potential for benefit and the treatment was discontinued. She was fitted with bilateral metal, double upright ankle-foot orthoses (AFOs) and received gait training using a platform walker. The occupational therapist fitted her with bilateral wrist splints that were custom-molded and padded. She learned the use of appropriate adaptive equipment for ADL training. For sensory reeducation, the occupational therapist used a variety of materials including rice, shaving cream, gel, and powder. The patient required Isotoner gloves, Ace wraps, and elastic stockings to control the edema in her extremities. Neuropsychology personnel evaluated and monitored the patient’s cognitive status and mood for signs of recurrent depression. To prevent contractures, the nursing staff applied bilateral lower extremity boots when the patient was in bed and also monitored her skin integrity. One week after admission, the patient developed questionable electrocardiographic changes and was transferred to the acute setting for three days; when no evidence of a cardiac event was found, she was returned to the rehabilitation center. Three weeks
Arch
Phys Med
Rehabil
Vol78,
January
1997
Mukand
later, she had increased right ankle edema and ecchymosis, but denied having pain or a history of trauma. X-ray showed a small avulsion fracture of the tip of the right lateral malleolus and a healing fracture of the right fifth proximal phalanx. Because of her profound sensory deficits in the distal lower extremities, the most likely etiology of these fractures was trauma during transfers or ambulation. She was restricted to non-weight-bearing status for 1 week and then returned to full weight-bearing with her bilateral AFOs without experiencing problems. She also required a urology consultation for a bloody urethral discharge and a history of recurrent urinary tract infections. She was diagnosed with urethral prolapse and corrective surgery was planned. The patient progressed steadily throughout her rehabilitative course despite these minor setbacks. Upper limb strength improved approximately one muscle grade from admission and there was some functional return of the left wrist and hand for gross motor tasks. Her sensory function remained severely compromised, but with adaptive equipment she could feed herself and required only minimal assistance for dressing and bathing. Her transfer ability improved from requiring maximum assistance at admission to needing only supervision. She was completely independent in bed mobility. She could ambulate safely with her AFOs and a platform walker for 100 feet with only contact guard assistance. Her overall FIM score rose from 61 at admission to 81 at discharge. She still required maximal assistance for bathing and for dressing the lower extremities. Toileting and toilet transfers were with supervision. Because of several factors, she was unable to return to her home. Although her family members were supportive, they were unable to provide supervision and there were concerns for her safety. In addition, her home would have required major architectural modifications to become wheelchair-accessible. Therefore, she was transferred to a skilled nursing facility for additional rehabilitation.
DISCUSSION This case illustrates the various issues involved in the rehabilitative management of a patient with polyarteritis, including some of the important complications associated with this condition. Peripheral nerves may be involved in 50% to 70% of patients with polyarteritis and, as in this case, may lead to the initial presentation.2,6.9 Arteritis of the vasa nervorum located in the epineurium results in widespread nerve fiber degeneration. Unlike diabetic peripheral neuropathy, the upper and lower extremities are equally likely to be affected. Pain and paresthesias in a particular peripheral nerve distribution precede a motor deficit. A multiple mononeuropathy ensues, often progressing to a symmetrical polyneuropathy that involves motor function and every sensory modality. As with any peripheral neuropathy, the principles of skin integrity, hygiene, and joint protection are of paramount importance. Effective patient education and adequate control of dependent edema to prevent skin breakdown are especially important, because acute and often permanent sensory losses occur. Stress fractures of the feet may or may not be avoidable, and should be suspected if there is new onset of edema or ecchymosis because pain may not be a symptom. Proper orthotic devices may reduce the incidence of this complication. Metal and leather AFOs are preferable for optimal support, skin integrity, and durability if the patient has the potential to be ambulatory. The extra weight may also help increase proprioceptive feedback to the patient’s less involved proximal joints and thus improve gait training. With upper limb involvement, wrist splints are an essential
REHABILITATION
FOR POLYARTERITIS,
adjunct to occupational therapy for protecting anesthetic extremities and improving proprioception. In addition, forearm troughs attached to a front-wheeled walker can result in profound improvement in ambulation. They allow more weightbearing as the strong proximal shoulder depressors can compensate for the distal motor deficits. As a result, patients gain more confidence and control of ambulation. The heart is affected in up to 75% of cases with PA studied at autopsy,” and the findings include cardiac hypertrophy, pericarditis, coronary arteritis, and myocardial infarction.” Notably, cardiac involvement is often clinically silent, and one study of 165 patients found only an 18% incidence of cardiac failure.6 Despite this, heart failure contributes to death in up to 50% of the cases; signs of cardiac compromise must be investigated, as was done when our patient had some questionable electrocardiographic changes. Patients should be carefully monitored for cardiac complications. Renal involvement results in nearly 65% of the deaths associated with classic PA.‘,” Autopsy studies indicate that up to 85% of cases may be affected with either renal polyarteritis (60%) or glomerulonephritis (25%).“.r3 The clinical manifestation of proteinuria is common and occasionally a nephrotic syndrome develops. Microscopic and even macroscopic hematuria may occur with progressive renal failure due to glomerulonephritis. Secondary renal hypertension is present in about 30% of the cases, but is usually amenable to pharmacologic therapy.6 Our patient did not have any serious renal complications; her bloody urethral discharge was unrelated to the arteritis. A thorough evaluation of genitourinary complaints in this patient population is always warranted, given the potential for fatal renal complications.
working with these patients, one must remain vigilant for the serious cardiac and renal complications of this disease and be prepared to make appropriate management decisions when necessary. I.
2. 3. 4. 5. 6. I.
8. 9. 10.
CONCLUSION Polyarteritis is an uncommon disease with the potential for multiple systemic complications. Peripheral neuropathy with associated motor weakness affects more than half of the patients, making them candidates for rehabilitative services. Unlike other polyneuropathies, such as Guillain-Barre syndrome, neuropathy related to PA has a much poorer prognosis, but this does not preclude successful rehabilitative outcomes. When
91
Mukand
11. 12. 13.
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Arch
Phys Med
Rehabil
Vol78,
January
1997