Department of Dermatology, The University of Tokyo Graduate School of Medicine, Tokyo, Japan.
Front Immunol. 2022 Jul 28;13:938785. doi: 10.3389/fimmu.2022.938785. eCollection 2022.
Systemic sclerosis (SSc) is a rare intractable systemic disease that causes fibrosis and vasculopathy against a background of autoimmune abnormalities. Although the etiology is not yet fully understood, the type of autoantibodies detected in SSc is closely associated with disease severity and prognosis, supporting that those autoimmune abnormalities play an important role in the pathogenesis of SSc. Although the direct pathogenicity of autoantibodies found in SSc is unknown, many previous studies have shown that B cells are involved in the development of SSc through a variety of functions. Furthermore, a number of clinical studies have been conducted in which B-cell depletion therapy has been tried for SSc, and many of these studies have found B-cell depletion therapy to be effective for SSc. However, the involvement of B cells in pathogenesis is complex, as they not only promote inflammation but also play an inhibitory role. This article outlines the role of B cells in the development of SSc, including the latest research.
系统性硬化症(SSc)是一种罕见的难治性系统性疾病,以自身免疫异常为背景引起纤维化和血管病变。虽然病因尚未完全阐明,但在 SSc 中检测到的自身抗体类型与疾病严重程度和预后密切相关,支持这些自身免疫异常在 SSc 的发病机制中起重要作用。尽管 SSc 中发现的自身抗体的直接致病性尚不清楚,但许多先前的研究表明,B 细胞通过多种功能参与 SSc 的发生。此外,已经进行了许多临床研究,尝试用 B 细胞耗竭疗法治疗 SSc,其中许多研究发现 B 细胞耗竭疗法对 SSc 有效。然而,B 细胞在发病机制中的作用是复杂的,因为它们不仅促进炎症,而且还起抑制作用。本文概述了 B 细胞在 SSc 发病机制中的作用,包括最新的研究。