炎性肌病——最新进展。

Inflammatory muscle disease - An update.

机构信息

Johns Hopkins University School of Medicine, 5501 Hopkins Bayview Circle, Suite 1B.1, Baltimore, MD 21224 USA.

Johns Hopkins University School of Medicine, 5200 Eastern Avenue, MFL Building, Center Tower Suite 4500, Baltimore, MD 21224 USA.

出版信息

Best Pract Res Clin Rheumatol. 2020 Feb;34(1):101484. doi: 10.1016/j.berh.2019.101484. Epub 2020 Feb 8.

Abstract

Idiopathic inflammatory myopathies (IIM) are a heterogeneous group of inflammatory myopathies whose common feature is immune-mediated muscle injury. There are distinct subgroups including dermatomyositis (DM), polymyositis (PM), inclusion body myositis, and immune-mediated necrotizing myopathy. Antisynthetase syndrome is also emerging as a distinct subgroup with its unique muscle histopathological characteristic of perifascicular necrosis. While the newly updated EULAR/ACR Classification Criteria for IIM have brought advancements in diagnosis and the exclusion of mimickers, the use of only one autoantibody in the derivation of the schema limits its use. Similarly, while the advent of multiple novel therapeutics in the treatment of myositis has been exciting, it has also highlighted the scarcity of validated outcome measures. The purpose of our review is to highlight the updated classification criteria of myositis, newly reported clinical phenotypes associated with myositis autoantibodies, the measurement of outcomes, and emerging treatments in the field.

摘要

特发性炎性肌病(IIM)是一组具有异质性的炎性肌病,其共同特征是免疫介导的肌肉损伤。存在明显的亚组,包括皮肌炎(DM)、多发性肌炎(PM)、包涵体肌炎和免疫介导的坏死性肌病。抗合成酶综合征也是一个明显的亚组,其独特的肌肉组织病理学特征是肌纤维旁坏死。虽然最近更新的 EULAR/ACR IIM 分类标准在诊断和排除类似疾病方面取得了进展,但该标准仅使用一种自身抗体来推导,限制了其应用。同样,虽然新型治疗药物在肌炎治疗中的应用令人兴奋,但也凸显了验证后的疗效指标的缺乏。我们的综述旨在强调肌炎的最新分类标准、与肌炎自身抗体相关的新报告的临床表型、疗效的测量以及该领域的新兴治疗方法。

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