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  • Review Article
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Autoimmune myopathies: autoantibodies, phenotypes and pathogenesis

Nature Reviews Neurology volume 7, pages 343–354 (2011)Cite this article

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Abstract

The different autoimmune myopathies—for example, dermatomyositis, polymyositis, and immune-mediated necrotizing myopathies (IMNM)—have unique muscle biopsy findings, but they also share specific clinical features, such as proximal muscle weakness and elevated serum levels of muscle enzymes. Furthermore, around 60% of patients with autoimmune myopathy have been shown to have a myositis-specific autoantibody, each of which is associated with a distinct clinical phenotype. The typical clinical presentations of the autoimmune myopathies are reviewed here, and the different myositis-specific autoantibodies, including the anti-synthetase antibodies, dermatomyositis-associated antibodies, and IMNM-associated antibodies, are discussed in detail. This Review also focuses on a newly recognized form of IMNM that is associated with statin use and the production of autoantibodies that recognize 3-hydroxy-3-methylglutaryl-coenzyme A reductase, the pharmacological target of statins. The contribution of interferon signaling to the development of dermatomyositis and the potential link between malignancies and the initiation of autoimmune myopathies are also assessed.

Key Points

  • The autoimmune myopathies include dermatomyositis, polymyositis, and immune-mediated necrotizing myopathies

  • Autoimmune muscle disease typically presents with subacute onset of proximal muscle weakness, elevated muscle enzyme levels, an irritable myopathy on electromyography, and inflammation and/or necrosis of myofibers on muscle biopsy

  • The majority of patients with autoimmune myopathy have one of the myositis-specific autoantibodies, each of which is associated with a distinct clinical phenotype

  • Statin-triggered autoimmune myopathy is a newly recognized form of muscle disease that is associated with autoantibodies recognizing 3-hydroxy-3-methylglutaryl-coenzyme A reductase, the pharmacological target of statins

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Figure 1: Muscle biopsies from patients with polymyositis, dermatomyositis or immune-mediated necrotic myopathy.
Figure 2: Thigh MRI from a patient with dermatomyositis.
Figure 3: Gottron papules.
Figure 4: Hyperkeratotic lesions associated with antisynthetase syndrome.
Figure 5: Initiation and maintenance of autoantibody production.

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Acknowledgements

A. Mammen is supported by the NIH (grant K08-AR-054,783). C. P. Vega, University of California, Irvine, CA, is the author of and is solely responsible for the content of the learning objectives, questions and answers of the Medscape, LLC-accredited continuing medical education activity associated with this article.

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  1. Departments of Neurology and Medicine, Johns Hopkins Bayview Medical Center, Johns Hopkins Myositis Center, Mason F. Lord Building Center Tower, Suite 4500, Baltimore, USA, 21224, MD

    Andrew L. Mammen

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The author declares an association with the following organization: Johns Hopkins University. See the article online for full details of the relationship. The journal Chief Editor H. Wood and the CME questions author C. P. Vega declare no competing interests.

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Mammen, A. Autoimmune myopathies: autoantibodies, phenotypes and pathogenesis. Nat Rev Neurol 7, 343–354 (2011). https://doi.org/10.1038/nrneurol.2011.63

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