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首页> 外文期刊>Nature clinical practice. Rheumatology >Mechanisms of disease: autoantigens as clues to the pathogenesis of myositis.
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Mechanisms of disease: autoantigens as clues to the pathogenesis of myositis.

机译：疾病机理：自身抗原是肌炎发病机制的线索。

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摘要

Autoimmune inflammatory myopathies, referred to as myositis, comprise a heterogeneous group of chronic inflammatory muscle diseases that present with various clinical phenotypes, histologic changes and autoantibodies, resulting in progressive inflammatory muscle damage and weakness. In up to 20% of myositis patients, particularly those with dermatomyositis, there is an association with cancer that is most frequently diagnosed within 1 year of presentation of myositis. Accumulating data show that autoantibodies in myositis target a specific group of intracellular molecules that are not muscle-specific in their expression. The striking association between autoantibodies recognizing ubiquitously expressed molecules and distinct clinical phenotypes suggests that the target tissues themselves might regulate and shape the phenotype-specific immune response in myositis. Studies indicate that changes in phenotype-specific autoantigens, such as altered structure, enhanced expression, and acquisition of adjuvantproperties during various forms of cellular stress, apoptosis, and transformation, might be mechanistically important in this regard. This Review discusses these developments and highlights a central role of autoantigens themselves as a critical partner in driving autoimmune diseases, and the potential for their therapeutic manipulation. In addition, we will highlight insights that the cancer-autoimmunity interface in this group of diseases provides into the relationship between the anticancer immune response and autoimmune diseases.
机译：自身免疫性炎症性肌病，称为肌炎，包括一组异质性的慢性炎症性肌肉疾病，表现为各种临床表型，组织学变化和自身抗体，导致进行性炎症性肌肉损伤和无力。在多达20％的肌炎患者中，特别是患有皮肌炎的患者，与肌炎的关联最常在肌炎发作1年内被诊断出来。越来越多的数据表明，肌炎中的自身抗体靶向一组特定的细胞内分子，这些分子的表达不是肌肉特异性的。识别普遍表达的分子的自身抗体与独特的临床表型之间的惊人联系表明，靶组织自身可能调节和塑造肌炎中表型特异性免疫反应。研究表明，在各种形式的细胞应激，细胞凋亡和转化过程中，表型特异性自身抗原的改变（例如结构改变，表达增强和获得佐剂特性）可能在机械方面很重要。这篇综述讨论了这些发展，并强调了自身抗原本身在驱动自身免疫性疾病中的重要作用，以及它们在治疗上的潜力。此外，我们将重点介绍这一类疾病中的癌症-自身免疫界面提供了抗癌免疫反应与自身免疫疾病之间关系的见解。

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