Muscle Disease Section, National Institute of Arthritis and Musculoskeletal and Skin Diseases, National Institutes of Health, 50 South Drive, Room 1141, Building 50, MSC 8024, Bethesda, MD, 20892, USA.
Department of Neurology, Johns Hopkins University School of Medicine, Baltimore, MD, USA.
Curr Rheumatol Rep. 2024 Dec;26(12):421-430. doi: 10.1007/s11926-024-01164-7. Epub 2024 Sep 24.
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 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.
本文旨在全面而系统地综述自身免疫性肌病，重点关注自身抗体作用的最新研究进展。我们将首先探讨与肌炎相关的风险因素和触发因素。接下来，深入研究自身抗体如何导致疾病发病机制。最后，探讨治疗策略的最新进展及其对我们理解肌炎发病机制的影响。
最近的研究揭示了肌炎特异性自身抗体可以浸润肌肉细胞并破坏其靶自身抗原的功能，在疾病发病机制中起着至关重要的作用。治疗方面的重要进展包括 CD19 CAR-T 细胞疗法、JAK-STAT 抑制剂以及皮肌炎中针对 I 型干扰素途径的新策略。此外，补体抑制剂在治疗免疫介导性坏死性肌炎中的无效性挑战了对疾病机制的既定观点。自身免疫性肌病是一组受特定自身抗体显著影响的疾病，这些抗体驱动疾病发病机制。本综述强调了自身抗体研究在加深对这些疾病认识中的关键作用，并讨论了针对关键致病途径的最新治疗进展。
