Evaluation and Treatment of the Idiopathic Inflammatory Myopathies

BACKGROUND The idiopathic inflammatory myopathies are clinically, histologically, and pathogenically distinct. In addition, the prognoses and response to treatment of the various forms of inflammatory myopathy are different. Most patients with inflammatory myopathy require aggressive immunosuppressive treatment. Clinicians need to be aware of the treatment options, which can be challenging and associated with various complications. REVIEW SUMMARY In this article, we review the clinical, electrophysiological, laboratory, and histological features of the major categories of idiopathic inflammatory myopathy (dermatomyositis, polymyositis, and inclusion body myositis) and some of the less common disorders (eosinophilic polymyositis, granulomatous or giant cell myositis, myositis secondary to sarcoidosis and Behcet's disease, and focal myositis). Recent advances in understanding the pathogenesis of these disorders are discussed. In addition, we detail our approach to diagnosing and managing patients with inflammatory myopathy. CONCLUSIONS Diagnosis and management of patients with inflammatory myopathy can be difficult. Better under-standing of the pathogenic basis for these distinct myositises hopefully will result in better treatment options. Prospective, double-blind, controlled trials are necessary to define the best treatment options. (THE NEUROLOGIST 6:267-287, 2000)