Centre for Immunology and Infectious Disease, Barts and The London School of Medicine and Dentistry, Blizard Institute, London, UK.
Clin Exp Immunol. 2012 Jan;167(1):7-14. doi: 10.1111/j.1365-2249.2011.04460.x.
Systemic lupus erythematosus (SLE) and Sjögren's syndrome are autoimmune disorders which are characterized by a disturbed B cell homeostasis which leads ultimately to dysfunction of various organs. One of the B cell subsets that appear in abnormal numbers is the population of transitional B cells, which is increased in the blood of patients with SLE and Sjögren's syndrome. Transitional B cells are newly formed B cells. In mice, transitional B cells undergo selection checks for unwanted specificity in the bone marrow and the spleen in order to eliminate autoreactive B cells from the circulating naive B cell population. In humans, the exact anatomical compartments and mechanisms of the specificity check-points for transitional B cells remain unclear, but appear to be defective in SLE and Sjögren's syndrome. This review aims to highlight the current understanding of transitional B cells and their defects in the two disorders before and after B cell-targeted therapies.
系统性红斑狼疮(SLE)和干燥综合征是自身免疫性疾病,其特征是 B 细胞稳态紊乱,最终导致各种器官功能障碍。异常数量出现的 B 细胞亚群之一是过渡性 B 细胞群体,SLE 和干燥综合征患者的血液中过渡性 B 细胞数量增加。过渡性 B 细胞是新形成的 B 细胞。在小鼠中,过渡性 B 细胞在骨髓和脾脏中进行选择检查,以消除循环中的幼稚 B 细胞群体中的自身反应性 B 细胞。在人类中,过渡性 B 细胞特异性检查点的确切解剖隔室和机制仍不清楚,但在 SLE 和干燥综合征中似乎存在缺陷。这篇综述旨在强调在 B 细胞靶向治疗之前和之后,过渡性 B 细胞及其缺陷在这两种疾病中的最新认识。