The idiopathic inflammatory myopathies (IIM) are a group of autoimmune diseases resulting from inflammation of muscle and manifesting as weakness, though a range of extra-muscular manifestations are observed. These are often correlated closely with disease subtype and the presence of myositis-specific/myositis-associated antibodies. IIM are notoriously difficult to treat and often refractory to glucocorticoid therapy and synthetic immunosuppressants. Both the innate and adaptive immune systems are implicated in the pathogenesis of IIM. A growing understanding of the key cytokines as well as the cell-mediated and antibody effectors of disease has identified multiple potential targets for biologic therapy. The most widely used of these is B-cell depletion via rituximab though the tumour necrosis factor inhibitors and other biologic therapies used in diseases such as rheumatoid arthritis, systemic lupus erythematosus and multiple sclerosis have also been trialled. This review summarises the literature thus far on biologic therapy in IIM, highlighting both the significant trials that influence current treatment regimens and also the continuing need for further research to inform more effective therapies.

中文翻译：

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生物疗法在特发性炎症性肌病中。

特发性炎症性肌病（IIM）是一组自身免疫性疾病，由肌肉炎症引起并表现为虚弱，尽管观察到一系列肌肉外表现。这些通常与疾病亚型以及肌炎特异性/肌炎相关抗体的存在密切相关。众所周知，IIM难以治疗，并且通常对糖皮质激素治疗和合成免疫抑制剂无效。先天免疫系统和适应性免疫系统均与IIM的发病机制有关。对关键细胞因子以及疾病的细胞介导和抗体效应子的日益了解，已经确定了生物治疗的多个潜在靶标。这些药物中使用最广泛的是通过利妥昔单抗清除B细胞，尽管也已经尝试了肿瘤坏死因子抑制剂和其他用于风湿性关节炎，系统性红斑狼疮和多发性硬化症等疾病的生物疗法。这篇综述总结了迄今为止有关IIM中生物疗法的文献，着重介绍了影响当前治疗方案的重要试验，以及继续进行进一步研究以提供更有效疗法的持续需求。