Sontheimer Richard D
University of Utah School of Medicine, Salt Lake City, Utah, USA.
Ann Transl Med. 2017 Apr;5(7):160. doi: 10.21037/atm.2017.03.94.
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).
艾伦巴赫及其同事最近首次报告了一项有趣研究的结果,该研究针对患有和未患有循环MDA5自身抗体(抗MDA5)的成年皮肌炎(DM)患者肌肉活检组织中的组织病理学、免疫病理学和基因表达差异进行了研究。抗MDA5最初是在临床定义的DM患者亚组中发现的,这些患者的疾病主要在皮肤中异常长时间地表现,而无伴随的肌肉无力[即“临床无肌病性皮肌炎”(CADM)],并且有患急性、快速进展型间质性肺病(ILD)的风险。作为一名在美国的学术皮肤科医生,我长期以来一直关注CADM亚组,我想分享我对艾伦巴赫及其同事这项工作结果的看法,并为该领域的进一步研究提供一些建议。但为了最有效地做到这一点,我首先想回顾一下CADM的临床概念及其与抗MDA5抗体产生和一种潜在致命形式的(ILD)的关联。