Dermatomyositis and polymyositis: Clinical presentation, autoantibodies, and pathogenesis.
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.
Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, Maryland, USA. amammen@jhmi.edu
Andrew L
AL
Mammen
D000328:Adult; D017668:Age of Onset; D000818:Animals; D001323:Autoantibodies / Q000097:blood*; D001706:Biopsy; D003882:Dermatomyositis / Q000175:diagnosis* / Q000235:genetics / Q000276:immunology / Q000473:pathology; D006801:Humans; D007125:Immunogenetics; D007249:Inflammation / Q000473:pathology; D008279:Magnetic Resonance Imaging; D051379:Mice; D018482:Muscle, Skeletal / Q000473:pathology; D009133:Muscular Atrophy / Q000473:pathology; D009336:Necrosis; D017285:Polymyositis / Q000175:diagnosis* / Q000276:immunology / Q000473:pathology; D012867:Skin / Q000276:immunology / Q000473:pathology
D001323:Autoantibodies