Groener Marwin, Paik Julie J
Division of Rheumatology, Department of Medicine, Johns Hopkins University School of Medicine, Baltimore, MD, United States.
Front Immunol. 2025 Jul 17;16:1581323. doi: 10.3389/fimmu.2025.1581323. eCollection 2025.
Autoantibodies play a crucial role in the diagnosis and clinical characterization of idiopathic inflammatory myopathies (IIM). These antibodies are categorized into myositis-specific autoantibodies (MSAs), which are unique to IIM, and myositis-associated autoantibodies (MAAs), which can be seen with other autoimmune diseases. Both plasmablasts and plasma cells contribute to the production of these autoantibodies. Current B cell-targeted therapies, such as rituximab, have shown promise in refractory IIM, although their limitations - particularly in targeting plasmablasts and plasma cells - highlight the need for alternative agents with greater efficacy. This review discusses the evolving landscape of B cell and plasma cell-targeted immunotherapies in IIM, including next-generation anti-CD20 antibodies, BAFF inhibition, anti-CD19 CAR-T cells, BCMA-targeted therapies, and anti-CD38 antibodies. Most studies on the use of these novel treatment strategies in IIM have reported positive outcomes, although the number of patients treated is small. While these therapies represent a paradigm shift, further randomized clinical trials are needed to identify optimal strategies for IIM management and establish long-term safety and efficacy.
自身抗体在特发性炎性肌病（IIM）的诊断和临床特征中起着关键作用。这些抗体分为肌炎特异性自身抗体（MSA），它是IIM所特有的，以及肌炎相关自身抗体（MAA），后者在其他自身免疫性疾病中也可见。浆母细胞和浆细胞都参与了这些自身抗体的产生。目前针对B细胞的疗法，如利妥昔单抗，在难治性IIM中已显示出前景，尽管其局限性——特别是在靶向浆母细胞和浆细胞方面——凸显了对具有更高疗效的替代药物的需求。本综述讨论了IIM中针对B细胞和浆细胞的免疫疗法的发展态势，包括新一代抗CD20抗体、BAFF抑制、抗CD19嵌合抗原受体T细胞、靶向BCMA的疗法以及抗CD38抗体。关于在IIM中使用这些新型治疗策略的大多数研究都报告了积极的结果，尽管接受治疗的患者数量较少。虽然这些疗法代表了一种范式转变，但仍需要进一步的随机临床试验来确定IIM管理的最佳策略，并确立长期的安全性和疗效。
