肌炎特异性自身抗体有助于多肌炎的诊断

作者：Z. Betteridge，et al.

翻译：中日友好医院吴东海

摘要：特发性炎性肌病以肌无力、皮肤病和内脏器官受累为特点。已知自身免疫在肌炎的发病中起作用，针对细胞内重要蛋白的肌炎特异性自身抗体被视为有助于患者诊断的关键生物标志物。近年来，在成人和青少年患者中，已确定了一批新的肌炎抗体，包括抗TIF1、抗NXP2、抗MDA5、抗SAE、抗HMGCR和抗N1A。这些自身抗体与不同的临床表现相关，重要的是也在包涵体肌炎、他汀类药物诱发性肌炎、临床无肌病性皮肌炎和幼年性肌炎患者（以前认为自身抗体阴性）中发现。在本文中，我们将描述主要的肌炎特异性和肌炎相关自身抗体，及其在不同年龄和种族群中的频率和临床相关性。我们还将讨论特异性肌炎抗体滴度与病程临床指标之间相关性的初步研究，展示肌炎自身抗体作为疾病诊断和预后标志物的价值。

附原文：The idiopathic inflammatory myopathies are characterized by muscle weakness, skin disease and internal organ involvement. Autoimmunity is known to have a role in myositis pathogenesis, and myositis-specific autoantibodies, targeting important intracellular proteins, are regarded as key biomarkers aiding in the diagnosis of patients. In recent years, a number of novel myositis autoantibodies including anti-TIF1, anti-NXP2, anti-MDA5, anti-SAE, anti-HMGCR and anticN1A have been identified in both adult and juvenile patients. These autoantibodies correlate with distinct clinical manifestations and importantly are found in inclusion body, statin-induced, clinically amyopathic and juvenile groups of myositis patients, previously believed to be mainly autoantibody negative. In this review, we will describe the main myositis-specific and myositis associated autoantibodies and their frequencies and clinical associations across different ages and ethnic groups. We will also discuss preliminary studies investigating correlations between specific myositis autoantibody titres and clinical markers of disease course, collectively demonstrating the utility of myositis autoantibodies as both diagnostic and prognostic markers of disease.

引自：Z. Betteridge & N. McHugh. Myositis-specific autoantibodies: an important tool to support diagnosis of myositis. J Intern Med, 2016 Jul;280(1):8-23. doi: 10.1111/joim.12451. Epub 2015 Nov 25.