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Inflammatory Myopathies

Amato, Anthony A. MD, FAAN; Greenberg, Steven A. MD

doi: 10.1212/
Review Articles

Purpose of Review To discuss the clinical, laboratory, and histopathologic features and presumed pathogenic mechanisms of the four major categories of idiopathic inflammatory myopathy, namely dermatomyositis, polymyositis, immune-mediated necrotizing myopathy, and inclusion body myositis.

Recent Findings Dermatomyositis, polymyositis, necrotizing myopathy, and inclusion body myositis are clinically, histologically, and pathogenically distinct. Polymyositis is a T cell–mediated disorder directed against muscle fibers. The pathogenesis of dermatomyositis, necrotizing myopathy, and inclusion body myositis are unknown. Dermatomyositis, polymyositis, and necrotizing myopathy are generally, but not always, responsive to immunosuppressive therapy, in contrast to inclusion body myositis, which is generally refractory to therapy.

Summary The pattern of muscle weakness, other clinical features (eg, rash, concurrent interstitial lung disease), laboratory features (creatine kinase, autoantibodies), and muscle biopsies are useful in distinguishing subtypes of inflammatory myopathy and in guiding treatment. More research is necessary to unravel the exact pathogenic bases of these myopathies and identify better treatments.

Address correspondence to Dr Anthony A. Amato, Department of Neurology, Brigham and Women’s Hospital and Harvard Medical School, 75 Francis Street, Boston, MA 02115,

Relationship Disclosure: Dr Amato has served on the medical advisory board of Biogen Idec, as a consultant for MedImmune, LLC, and provided expert testimony for litigation on a neuropathy case. Dr Amato serves as an associate editor of Neurology. Dr Greenberg has served as a consultant for aTyr Pharma, as an expert witness in litigation pertaining to copper deficiency, and is supported by a grant from the Muscular Dystrophy Association.

Unlabeled Use of Products/Investigational Use Disclosure: Drs Amato and Greenberg discuss therapies for the treatment of myositis, all of which are unlabeled.

© 2013 American Academy of Neurology
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