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Dermatomyositis and Type 1 Interferons

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Abstract

Dermatomyositis is a poorly understood multisystem disease predominantly affecting skin and muscle. This review focuses on the potential role of a group of related cytokines, the type 1 interferons, in the pathogenesis of dermatomyositis. Type 1 interferon–inducible transcripts and proteins are uniquely elevated in dermatomyositis muscle compared with all other muscle diseases studied to date. The endothelial cell tubuloreticular inclusions present in affected dermatomyositis muscle are biomarkers of type 1 interferon exposure. The cell-poor lichenoid reaction in skin with predominant involvement of the basal epidermal cell layer and its topologic equivalent in muscle, perifascicular atrophy, may be lesions that develop directly in response to type 1 interferon signaling.

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Acknowledgments

This work was supported by grants to Dr. Greenberg from the National Institutes of Health (NIH R01NS43471 and R21NS057225) and the Muscular Dystrophy Association (MDA3878).

Disclosure

Dr. Greenberg has served as a consultant regarding clinical trial planning for MedImmune, LLC and has a sponsored research agreement with MedImmune, LLC. He is also an inventor of intellectual property pertaining to myositis diagnostics.

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Authors and Affiliations

  1. Department of Neurology, Division of Neuromuscular Disease, Brigham and Women’s Hospital, 75 Francis Street, Boston, MA, 02115, USA

    Steven A. Greenberg

  2. Children’s Hospital Informatics Program, Harvard Medical School, 75 Francis Street, Boston, MA, 02115, USA

    Steven A. Greenberg

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  1. Steven A. Greenberg
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Correspondence to Steven A. Greenberg.

Additional information

A second publication [Nakashima et al Rheumatology 2010;49:433–40] has independently identified IFIH1 as a dermatomyositis-specific autoantigen.

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Greenberg, S.A. Dermatomyositis and Type 1 Interferons. Curr Rheumatol Rep 12, 198–203 (2010). https://doi.org/10.1007/s11926-010-0101-6

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