Abstract
Polymyositis is a rare autoimmune disease, characterized by inflammation and degeneration of the skeletal muscles. If the skin is involved it is called dermatomyositis (Figure 22.1a, b, c). The incidence is 2–6 patients per 1 million inhabitants. In polymyositis, clonally expanded CD8+ cytotoxic T cells invade muscle fibers that express MHC class I antigens, which leads to fiber necrosis via the perforin pathway. Dermatomyositis is a microangiopathy affecting skin and muscle; activation and deposition of complement causes lysis of endomysial capillaries and muscle ischemia. The causative autoantigen has not yet been identified.
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Bibliography
Douglas WW, Tazelaar HD, Hartman TE, Hartman RP, Decker PA, Schroeder DR, Ryu JH. Polymyositisdermatomyositis-associated interstitial lung disease. Am Rev Respir Crit Care Med 2001;164:1182–1185.
Fathi M, Dastmalchi M, Rasmussen E, Lundberg IE, Torling G. Interstitial lung disease, a common manifestation of newly diagnosed polymyositis and dermatomyositis. Ann Rheum Dis 2004;63:297–301.
Schnabel A, Hellmich B, Gross WL. Interstitial lung disease in polymyositis and dermatomyositis. Curr Rheumatol Rep 2005;7:99–105.
Dalakos MC, Hohlfeld R. Polymyosistis and dermatomyosistis. Lancet 2003;362:971–982.
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(2006). Polymyositis/Dermatomyositis and the Lung. In: Clinical Atlas of Interstitial Lung Disease. Springer, London. https://doi.org/10.1007/978-1-84628-326-0_22
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DOI: https://doi.org/10.1007/978-1-84628-326-0_22
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