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Dermatomyositis Clinical and Pathological Phenotypes Associated with Myositis-Specific Autoantibodies

  • Inflammatory Muscle Disease (I Lundberg, Section Editor)
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Abstract

Purpose of review

Dermatomyositis is an idiopathic inflammatory myopathy with a variety of systemic and cutaneous manifestations. The myositis-specific autoantibodies (MSAs) are associated with phenotypic features and provide a tool for sub-classification of dermatomyositis patients. This review focuses on recent work characterizing the clinical features that accompany the MSAs in dermatomyositis.

Recent findings

There is increasing recognition of the distinct clinical and pathological phenotypes associated with each MSA. Most of these features display considerable overlap between MSA groups. Despite this, there are notable differences between the typical combinations of cutaneous and systemic manifestations, response to therapy, prognosis, and disease sequelae that define each dermatomyositis MSA group.

Summary

The MSAs may ultimately improve diagnosis and sub-classification of dermatomyositis patients. However, more work is needed to understand the pathologic basis for much of the heterogeneity found within these subgroups.

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  1. Department of Dermatology, Stanford University School of Medicine, 450 Broadway Street, Redwood City, CA, 94063, USA

    Paige W. Wolstencroft & David F. Fiorentino

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Wolstencroft, P.W., Fiorentino, D.F. Dermatomyositis Clinical and Pathological Phenotypes Associated with Myositis-Specific Autoantibodies. Curr Rheumatol Rep 20, 28 (2018). https://doi.org/10.1007/s11926-018-0733-5

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