Autoantibodies in Polymyositis and Dermatomyositis
Autoantibodies in Polymyositis and Dermatomyositis
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DOI：
10.1007/s11926-013-0335-1
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发表时间：
2013-06-01
影响因子：
5
通讯作者：
Doria, Andrea
中科院分区：
文献类型：
--
作者：
Ghirardello, Anna;Bassi, Nicola;Doria, Andrea
炎性肌病是一组获得性疾病，其特征在于主要涉及骨骼肌的免疫组织化学过程。根据最近的分类标准，已经确定了四种主要疾病：多发性肌炎（PM）、皮肌炎（DM）、散发性包涵体肌炎（IBM）和坏死性自身免疫性肌炎（NAM）。在大多数肌炎患者的血清中可以发现自身抗体。肌炎特异性自身抗体（MSA）是肌炎谱内非常特异性疾病实体的标志物，并且是参与蛋白质合成关键过程的靶蛋白。肌炎自身抗原包括明确定义的氨酰-tRNA合成酶、Mi-2解旋酶/组蛋白脱乙酰酶蛋白复合物和信号识别颗粒（SRP）核糖核蛋白，以及新的靶点，如TIF 1-γ、MDA 5、NXP 2、SAE和HMGCR。最近的研究表明，自身抗原驱动肌肉中的B细胞抗原特异性免疫应答。有趣的是，与正常相比，证实了PM和DM患者肌肉活检中再生纤维中Jo-1和Mi-2的表达增加。在肿瘤组织中观察到肌炎自身抗原上调，因此代表了肌炎中癌症和自身免疫之间的潜在联系。非免疫机制似乎参与了炎症性肌病的发病机制;最近有报道称，肌炎患者在异常肌肉再生和炎症反应中诱导内质网应激反应。本文综述了主要自身抗体在肌炎中的临床和病理生理作用。
Inflammatory myopathies are a group of acquired diseases, characterized by immunoflogistic processes primarily involving the skeletal muscle. According to recent classification criteria, four major diseases have been identified: polymyositis (PM), dermatomyositis (DM), sporadic inclusion body myositis (IBM), and necrotizing autoimmune myositis (NAM). Autoantibodies can be found in the sera of most patients with myositis. Myositis-specific autoantibodies (MSAs) are markers of very specific disease entities within the spectrum of myositis, and target proteins involved in key processes of protein synthesis. Myositis autoantigens comprise the well-defined aminoacyl-tRNA synthetases, the Mi-2 helicase/histone deacetylase protein complex, and the signal recognition particle (SRP) ribonucleoprotein, together with novel targets such as TIF1-gamma, MDA5, NXP2, SAE, and HMGCR. Recent studies suggest that autoantigens drive a B cell antigen-specific immune response in muscles. Interestingly, an increased expression of Jo-1 and Mi-2 in regenerating fibers in muscle biopsies from PM and DM patients compared to normal was demonstrated. Myositis autoantigen upregulation was observed in neoplastic tissues, thus representing a potential link between cancer and autoimmunity in myositis. Non-immunological mechanisms seem to participate to the pathogenesis of inflammatory myopathies; induction of endoplasmic reticulum stress response in response to abnormal muscle regeneration and inflammation has recently been reported in patients with myositis. This review article provides an update of new emerging insights about the clinical and pathophysiologic role of principal autoantibodies in myositis.