抗 MDA5 抗体谱在西方世界。
Anti-MDA5 Antibody Spectrum in Western World.
机构信息
Myositis Center and Division of Rheumatology and Clinical Immunology, Department of Medicine, University of Pittsburgh School of Medicine, Pittsburgh, PA, USA.
VA Pittsburgh Healthcare System, Pittsburgh, PA, USA.
出版信息
Curr Rheumatol Rep. 2018 Oct 31;20(12):78. doi: 10.1007/s11926-018-0798-1.
PURPOSE OF REVIEW
Anti-melanoma differentiation-associated gene 5 (anti-MDA5) is a novel and highly specific myositis-associated autoantibody, which defines a unique phenotype among patients with dermatomyositis (DM).
RECENT FINDINGS
Anti-MDA5 was originally characterized in Japan in DM patients with hallmark cutaneous features and no proximal muscle weakness and termed clinically amyopathic DM (CADM). Anti-MDA5 has characteristic cutaneous manifestations which include tender palmar papules and cutaneous ulcerations, along with an increased frequency of interstitial lung disease (ILD) that can be rapidly progressive (RPILD) and fatal. This review will highlight the clinical significance of anti-MDA5 autoantibodies in Caucasian DM patients.
目的综述
抗黑色素瘤分化相关基因 5（抗-MDA5）是一种新型的、高度特异性的肌炎相关自身抗体，它在皮肌炎（DM）患者中定义了一种独特的表型。
最近的发现
抗-MDA5 最初在日本 DM 患者中被描述，这些患者具有标志性的皮肤特征，没有近端肌无力，被称为临床无肌病性皮肌炎（CADM）。抗-MDA5 具有特征性的皮肤表现，包括触痛性掌部丘疹和皮肤溃疡，以及间质性肺病（ILD）的发生率增加，可迅速进展（RPILD）和致命。这篇综述将重点介绍抗-MDA5 自身抗体在高加索 DM 患者中的临床意义。
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