Recent Updates on the Pathogenesis of Inflammatory Myopathies.

IF 5.7 2区医学 Q1 RHEUMATOLOGY Current Rheumatology Reports Pub Date : 2024-12-01 Epub Date: 2024-09-24 DOI:10.1007/s11926-024-01164-7

Jon Musai, Andrew L Mammen, Iago Pinal-Fernandez

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Abstract

Purpose of review: This review aims to provide a comprehensive and updated overview of autoimmune myopathies, with a special focus on the latest advancements in understanding the role of autoantibodies. We will begin by examining the risk factors and triggers associated with myositis. Next, we will delve into recent research on how autoantibodies contribute to disease pathogenesis. Finally, we will explore the latest innovations in treatment strategies and their implications for our understanding of myositis pathogenesis.

Recent findings: Recent research has revealed that myositis-specific autoantibodies can infiltrate muscle cells and disrupt the function of their target autoantigens, playing a crucial role in disease pathogenesis. Significant advances in treatment include CD19 CAR-T cell therapy, JAK-STAT inhibitors, and novel strategies targeting the type 1 interferon pathway in dermatomyositis. Additionally, the ineffectiveness of complement inhibitors in treating immune-mediated necrotizing myositis has challenged established views on disease mechanisms. Autoimmune myopathies are a collection of disorders significantly influenced by specific autoantibodies that drive disease pathogenesis. This review highlights the critical role of autoantibody research in deepening our understanding of these conditions and discusses recent therapeutic advancements targeting key pathogenic pathways.

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炎症性肌病发病机制的最新进展。

综述的目的：本综述旨在提供有关自身免疫性肌病的全面最新概述，尤其侧重于了解自身抗体作用的最新进展。首先，我们将探讨与肌炎相关的风险因素和诱发因素。接下来，我们将深入探讨有关自身抗体如何影响疾病发病机制的最新研究。最后，我们将探讨治疗策略的最新创新及其对我们了解肌炎发病机制的影响：最新研究发现，肌炎特异性自身抗体可浸润肌细胞并破坏其靶自身抗原的功能，在疾病发病机制中发挥着至关重要的作用。治疗方面的重大进展包括 CD19 CAR-T 细胞疗法、JAK-STAT 抑制剂和针对皮肌炎 1 型干扰素通路的新策略。此外，补体抑制剂在治疗免疫介导的坏死性肌炎方面效果不佳，这对疾病机制的既有观点提出了挑战。自身免疫性肌病是受特异性自身抗体显著影响的一系列疾病，这些抗体驱动着疾病的发病机制。本综述强调了自身抗体研究在加深我们对这些疾病的了解方面所起的关键作用，并讨论了针对关键致病途径的最新治疗进展。

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来源期刊

Current Rheumatology Reports RHEUMATOLOGY-

CiteScore

11.20

自引率

0.00%

发文量

期刊介绍： This journal aims to review the most important, recently published research in the field of rheumatology. By providing clear, insightful, balanced contributions by international experts, the journal intends to serve all those involved in the care and prevention of rheumatologic conditions. We accomplish this aim by appointing international authorities to serve as Section Editors in key subject areas such as the many forms of arthritis, osteoporosis and metabolic bone disease, and systemic lupus erythematosus. Section Editors, in turn, select topics for which leading experts contribute comprehensive review articles that emphasize new developments and recently published papers of major importance, highlighted by annotated reference lists. An international Editorial Board reviews the annual table of contents, suggests articles of special interest to their country/region, and ensures that topics are current and include emerging research. Commentaries from well-known figures in the field are also occasionally provided.