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特发性炎性肌病发病机制的最新进展。

An update on the pathogenesis of idiopathic inflammatory myopathies.

作者信息

Tiniakou Eleni

机构信息

Division of Rheumatology, Department of Medicine, Johns Hopkins University, Baltimore, Maryland, USA.

出版信息

Curr Opin Rheumatol. 2025 May 1;37(3):192-198. doi: 10.1097/BOR.0000000000001077. Epub 2025 Feb 12.

Abstract

PURPOSE OF REVIEW

As the question of the pathogenesis of inflammatory myopathies remains unanswered, there has been a significant effort in recent years to investigate various components of the innate and adaptive immune systems, with evidence pointing that they work together to initiate and propagate the autoimmune response. This review aims to explore recent advancements in understanding the mechanisms underlying myopathies.

RECENT FINDINGS

Recent research has concentrated on uncovering potential triggers, examining the role of immune cells, both lymphocytes and myeloids, and investigating the contribution of inflammatory mediators to the autoimmune response in inflammatory myopathies. Unsuccessful clinical trials helped reshape established hypotheses about pathogenesis, while genetic mutations offered clues to the disease's root causes. The pathogenic role of autoantibodies is being reconsidered based on transcriptional data. Repurposing existing medications to combat muscle fiber dysfunction is also emerging as a potential therapeutic approach.

SUMMARY

Our understanding of inflammatory myopathies has evolved significantly as our understanding of the disease has grown. Even though breakthroughs have been documented on the underlying mechanisms of myopathies, important questions remain unanswered.

摘要

综述目的

由于炎性肌病的发病机制问题仍未得到解答,近年来人们付出了巨大努力来研究先天性和适应性免疫系统的各个组成部分,有证据表明它们共同作用以启动和传播自身免疫反应。本综述旨在探讨在理解肌病潜在机制方面的最新进展。

最新发现

近期研究集中在揭示潜在触发因素、研究免疫细胞(包括淋巴细胞和髓样细胞)的作用以及探究炎症介质在炎性肌病自身免疫反应中的作用。临床试验的失败有助于重塑关于发病机制的既定假设,而基因突变则为疾病的根本原因提供了线索。基于转录数据,自身抗体的致病作用正在被重新审视。重新利用现有药物来对抗肌纤维功能障碍也正在成为一种潜在的治疗方法。

总结

随着我们对炎性肌病认识的增加,我们对其的理解有了显著进展。尽管在肌病的潜在机制方面已有突破记录,但重要问题仍未得到解答。

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