Polyarteritis nodosa presenting as polymyositis*

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Abstract

Background: Skeletal muscle involvement has been well documented in patients with polyarteritis nodosa (PAN), and symptoms referable to skeletal muscle are not uncommon. However, polymyositis as a mode of presentation of PAN is uncommon. This unusual presentation of PAN has been reported only once previously in the English literature. Objective: This study describes a patient who had diffuse weakness, myalgias, and markedly elevated serum creatinine phosphokinase, mimicking polymyositis. The literature dealing with the clinical aspects of muscle involvement in PAN is reviewed. Results: A 24-year-old man was admitted to the hospital with a 1-month history of fever, myalgia, and muscle weakness. Necrotizing vasculitis was shown on subsequent muscle biopsy, consistent with PAN. Literature review indicated that muscle involvement is common in PAN, as has been shown by the frequency of muscular symptoms and by histologic evidence obtained from both clinical and autopsy studies. Nineteen percent of patients with PAN had documented myopathy, and autopsy series have shown skeletal muscle involvement in 30% to 48% of cases. However, polymyositis as a mode of presentation of PAN is rare. We found only 1 other patient with PAN who had elevated creatinine phosphokinase and diffuse myopathy suggestive of polymyositis. Conclusions: PAN should be suspected in cases of focal or diffuse myopathy, especially in the context of a systemic disease. Biopsy of symptomatic muscles or EMG-directed biopsies can be helpful in establishing a diagnosis of PAN to allow the physician to provide early treatment. Semin Arthritis Rheum 31:377-383. Copyright 2002, Elsevier Science (USA). All rights reserved.

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Case report

A 24-year-old healthy white man began having myalgias, fever, sore throat, and muscular weakness 1 month before admission to the hospital. He received a course of oral antibiotics but showed no improvement. Thereafter, his symptoms progressed to include night sweats, weight loss of 12 pounds, and inability to rise from a chair or walk.

The patient's initial temperature was 100.1°F, heart rate 120 beats/min, blood pressure 118/68 mm Hg, and respiratory rate 26/min. He had an erythematous

Discussion

PAN is the prototypical necrotizing vasculitis, which involves small and medium muscular arteries. Kussmaul and Maier (2) first described the classic form of PAN in 1866. The pathologic hallmark of PAN is segmental necrotizing vasculitis, which leads to vessel wall damage, microaneurysms, and eventual end organ ischemia or necrosis (3).

PAN is a rare disease affecting men and women of all ages and races. The estimated prevalence of PAN is approximately 6.3 per 100,000 population. Prevalence

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    *

    Address reprint requests to Hugo E. Jasin, MD, Director, Division of Rheumatology and Clinical Immunology, University of Arkansas for Medical Sciences, Mail Slot 509, 4301 W. Markham, Little Rock, AR 72205. E-mail: [email protected]

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