Department of Rheumatology, Agios Andreas Hospital, Patras, Greece.
Department of Internal Medicine, Division of Rheumatology, Patras University Hospital, University of Patras Medical School, 26504 Rion, Patras, Greece.
Clin Rheumatol. 2021 Jul;40(7):2621-2631. doi: 10.1007/s10067-021-05665-z. Epub 2021 Mar 21.
Systemic sclerosis is a debilitating autoimmune disease with unknown pathogenesis. The clinical phenotype of fibrosis is preceded by vascular and immunologic aberrations. Adaptive immunity has been extensively studied in patients with the disease and B cells appear to be dysregulated. This is evident in peripheral blood B cell subsets, with activated effector B cells and impaired B regulatory function. In addition, B cells infiltrate target organs and tissues of patients with the disease, such as the skin and the lung, indicating a probable role in the pathogenesis. Impaired B cell homeostasis explains the rationale behind B cell therapeutic targeting. Indeed, several studies in recent years have shown that depletion of B cells appears to be a promising treatment alongside current established therapeutic choices, such as mycophenolate. In this review, B cell aberrations in animal models and human patients with systemic sclerosis will be presented. Moreover, we will also summarize current existing data regarding therapeutic targeting of the B cells in systemic sclerosis.
系统性硬化症是一种病因不明的使人虚弱的自身免疫性疾病。纤维化的临床表型之前是血管和免疫异常。适应性免疫在系统性硬化症患者中已经被广泛研究，而 B 细胞似乎失调。这在周围血 B 细胞亚群中表现明显，其中激活的效应 B 细胞和受损的 B 调节功能。此外，B 细胞浸润系统性硬化症患者的靶器官和组织，如皮肤和肺，表明其可能在发病机制中起作用。B 细胞稳态的破坏解释了 B 细胞治疗靶向的基本原理。事实上，近年来的几项研究表明，B 细胞耗竭似乎是一种有前途的治疗方法，与目前已确立的治疗选择（如霉酚酸酯）相结合。在这篇综述中，将介绍系统性硬化症动物模型和人类患者中的 B 细胞异常。此外，我们还将总结目前关于系统性硬化症中 B 细胞治疗靶向的现有数据。
