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-  2017 

MDA5 autoantibody—another indicator of clinical diversity in dermatomyositis

DOI: 10.21037/atm.2017.03.94

Keywords: Amyopathic dermatomyositis (ADM), classic dermatomyositis (CDM), clinically-amyopathic dermatomyositis (CADM, hypomyopathic dermatomyositis (HDM), interstitial lung disease (ILD), melanoma differentiation associated protein 5 (MDA5), MDA5 autoantibody (anti-MDA5), nitric oxide synthetase (NOS)

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Abstract:

Allenbach and colleagues have recently reported for the first time the results of an intriguing study of the histopathologic, immunopathologic and gene expression differences in muscle biopsy tissue from adult dermatomyositis (DM) patients who do and do not have circulating MDA5 autoantibodies (anti-MDA5). Anti-MDA5 were originally identified in a clinically-defined subset of DM patients whose disease was expressed predominately in the skin for unusually long periods of time without accompanying muscle weakness [i.e., “clinically-amyopathic DM” (CADM)] and were at risk for acute, rapidly-progressive form of interstitial lung disease (ILD). As an academic dermatologist in the United States of America (USA) having a career-long interest in the CADM subset, I would like to share my perspective on the results of the work by Allenbach and colleagues and offer some suggestions for additional study in this area. But to do so most effectively, I first would like to review the clinical concept of CADM and its association with anti-MDA5 antibody production and a potentially-fatal form of (ILD)

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