Liu Changyan, Zhang Panpan, Zhang Wen
Department of Rheumatology, The Second Hospital of Dalian Medical University, Dalian, 116023, China.
Department of Rheumatology, Peking Union Medical College Hospital, National Clinical Research Center for Dermatologic and Immunologic Diseases, Key Laboratory of Rheumatology and Clinical Immunology, Ministry of Education, Beijing, 100730, China.
J Transl Autoimmun. 2020 Mar 19;3:100047. doi: 10.1016/j.jtauto.2020.100047. eCollection 2020.
IgG4-related disease (IgG4-RD) is an immune-mediated inflammatory disorder in multiple organs, characterized by abundant infiltration of IgG4-positive plasmacytes and fibrosis in the involved organs. The precise pathogenic mechanism of IgG4-RD still remains unclear. Aberrant innate and adaptive immunity are considered as the main pathogenesis of IgG4-RD. Recent studies have shown that abnormal adaptive immune responses mediated by T helper type 2 cells, regulatory T lymphocytes, CD4 cytotoxic T lymphocytes, T follicular helper cells, T follicular regulatory cells, PD-1hiCXCR5-peripheral T helper cells and B cell subsets are involved in IgG4-RD. In addition to adaptive immune responses, innate immune responses play pathogenic roles in IgG4-RD. Macrophages, mast cells, basophils, complement, and plasmacytoid dendritic cells are activated to produce various kinds of cytokines in IgG4-RD. This review aims to summarize the most recent knowledge in the pathogenesis of IgG4-RD.
IgG4相关性疾病（IgG4-RD）是一种多器官免疫介导的炎症性疾病，其特征是受累器官中有大量IgG4阳性浆细胞浸润和纤维化。IgG4-RD的确切发病机制仍不清楚。异常的固有免疫和适应性免疫被认为是IgG4-RD的主要发病机制。最近的研究表明，由2型辅助性T细胞、调节性T淋巴细胞、CD4细胞毒性T淋巴细胞、滤泡辅助性T细胞、滤泡调节性T细胞、PD-1hiCXCR5外周辅助性T细胞和B细胞亚群介导的异常适应性免疫反应参与了IgG4-RD。除适应性免疫反应外，固有免疫反应在IgG4-RD中也发挥致病作用。在IgG4-RD中，巨噬细胞、肥大细胞、嗜碱性粒细胞、补体和浆细胞样树突状细胞被激活以产生各种细胞因子。本综述旨在总结IgG4-RD发病机制的最新知识。
