Yoshikawa Tomoe, Watanabe Tomohiro, Minaga Kosuke, Kamata Ken, Kudo Masatoshi
a Department of Gastroenterology and Hepatology , Kindai University Faculty of Medicine , Osaka , Japan.
Mod Rheumatol. 2019 Mar;29(2):219-225. doi: 10.1080/14397595.2018.1536364. Epub 2018 Dec 11.
IgG4-related disease (IgG4-RD) is a newly defined multi-organ disease proposed by Japanese physicians. IgG4-RD is characterized by elevated serum levels of IgG4 and massive infiltration of IgG4-expressing plasma cells in the affected organs. Recent studies have shown that abnormal adaptive immune responses mediated by T helper type 2 cells, regulatory T cells, follicular helper T cells, cytotoxic CD4 T cells, and plasmablasts are involved in IgG4-RD immunopathogenesis. In addition to adaptive immune responses, innate immune responses play pathogenic roles in IgG4-RD. Plasmacytoid dendritic cells (pDCs), M2 macrophages, and basophils are activated to produce various kinds of cytokines in IgG4-RD. Recent studies highlight the importance of type I IFN and IL-33 produced by pDCs in IgG4-RD immunopathogenesis.
IgG4相关疾病（IgG4-RD）是一种由日本医生新定义的多器官疾病。IgG4-RD的特征是血清IgG4水平升高以及受累器官中表达IgG4的浆细胞大量浸润。最近的研究表明，由2型辅助性T细胞、调节性T细胞、滤泡辅助性T细胞、细胞毒性CD4 T细胞和成浆细胞介导的异常适应性免疫反应参与了IgG4-RD的免疫发病机制。除了适应性免疫反应外，固有免疫反应在IgG4-RD中也发挥致病作用。浆细胞样树突状细胞（pDC）、M2巨噬细胞和嗜碱性粒细胞在IgG4-RD中被激活以产生各种细胞因子。最近的研究强调了pDC产生的I型干扰素和IL-33在IgG4-RD免疫发病机制中的重要性。
