Department of Dermatology, Groupe Hospitalier Paris Seine-Saint-Denis, AP-HP & INSERM UMR1125, Bobigny, France.
Immunotherapy. 2021 Jan;13(1):35-53. doi: 10.2217/imt-2020-0189. Epub 2020 Oct 12.
B-cells are major effector cells in autoimmunity since they differentiate into plasmocytes that produce pathogenic auto-antibody such as anti-desmoglein antibodies in pemphigus patients. Major advances were obtained using whole B-cell depleting therapies including anti-CD20 antibodies in refractory pemphigus patients that lead to rituximab approval in pemphigus patients in EU and USA. This review summarizes the data supporting the efficacy of rituximab in pemphigus and provides an overview of the reported immunological changes underlying its therapeutic action. Short and long-term remission in pemphigus is explained by the removal of autoreactive B-cells involved in the production of pathogenic IgG auto-antibodies and by enhancement of the appearance of regulatory B-cells that could maintain long term immune tolerance.
B 细胞是自身免疫中的主要效应细胞，因为它们分化为浆细胞，产生致病性自身抗体，如天疱疮患者中的抗桥粒芯糖蛋白抗体。在难治性天疱疮患者中使用全 B 细胞耗竭疗法，包括抗 CD20 抗体，取得了重大进展，这导致利妥昔单抗在欧盟和美国获得天疱疮患者的批准。这篇综述总结了支持利妥昔单抗在天疱疮中的疗效的数据，并概述了其治疗作用的报告免疫变化。天疱疮的短期和长期缓解是通过去除参与产生致病性 IgG 自身抗体的自身反应性 B 细胞和增强可能维持长期免疫耐受的调节性 B 细胞的出现来解释的。
