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Pathogenesis of Dermatomyositis: Role of Cytokines and Interferon

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Abstract

Dermatomyositis is a systemic autoimmune disease that primarily affects skeletal muscle, skin, and the lungs. Dermatomyositis is characterized by autoantibodies, tissue inflammation, parenchymal cell damage and death, and vasculopathy. This review focuses on recent advances regarding the role of cytokines and interferon in the pathogenesis of the disease. Evidence for the role of a particular cytokine is based on data showing dysregulated levels in tissue and/or blood; correlation with histopathologic or clinical markers of disease activity; and, rarely, clinical efficacy of targeted cytokine inhibitors. Many of the recent advances pertain to elucidation of the role of interferons in both muscle and skin disease in dermatomyositis. Although a great deal of progress has been made regarding the role of interferon in the disease, many critical questions remain unanswered.

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Disclosure

Dr. Fiorentino has served as a consultant for MedImmune. Drs. Kao and Chung reported no potential conflicts of interest relevant to this article.

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Authors and Affiliations

  1. Division of Immunology and Rheumatology, Stanford University School of Medicine, 1000 Welch Road, #203, Palo Alto, CA, 94304, USA

    Lily Kao

  2. Division of Immunology and Rheumatology, Stanford University School of Medicine, Palo Alto Veterans Affairs Health Care System, 3801 Miranda Avenue, Room B2-139, Palo Alto, CA, 94305, USA

    Lorinda Chung

  3. Department of Dermatology, Stanford University School of Medicine, 450 Broadway, C-234, Redwood, CA, 94063, USA

    David F. Fiorentino

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  1. Lily Kao
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  2. Lorinda Chung
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  3. David F. Fiorentino
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Correspondence to David F. Fiorentino.

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Kao, L., Chung, L. & Fiorentino, D.F. Pathogenesis of Dermatomyositis: Role of Cytokines and Interferon. Curr Rheumatol Rep 13, 225–232 (2011). https://doi.org/10.1007/s11926-011-0166-x

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