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Dermatomyositis and polymyositis

Clinical presentation, autoantibodies, and pathogenesis

Authors


Address for correspondence: Andrew L. Mammen, MD, PhD, Johns Hopkins University School of Medicine, Dept. of Neurology, Johns Hopkins Bayview, Johns Hopkins Myositis Center, Mason F. Lord Building Center Tower, Suite 4100, Baltimore, MD 21224. Voice: 410-550-6962; fax: 410-550-3542. amammen@jhmi.edu

Abstract

Dermatomyositis (DM) and polymyositis (PM) are autoimmune myopathies characterized clinically by proximal muscle weakness, muscle inflammation, extramuscular manifestations, and frequently, the presence of autoantibodies. Although there is some overlap, DM and PM are separate diseases with different pathophysiological mechanisms. Furthermore, unique clinical phenotypes are associated with each of the myositis-specific autoantibodies (MSAs) associated with these disorders. This review will focus on the clinical features, pathology, and immunogenetics of PM and DM with an emphasis on the importance of autoantibodies in defining unique phenotypes and, perhaps, as clues to help elucidate the mechanisms of disease.

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