IgG4相关性疾病（IgG4 related disease，IgG4-RD）是近年来新认识的以血清IgG4升高、组织中大量淋巴细胞和IgG4+浆细胞浸润、受累脏器增生和肿大为特点的自身免疫病。调节性B细胞（Breg）是一种能分泌IL-10并具有抑制和调节免疫应答反应的B细胞亚群，在多种自身免疫病中起重要作用。为探讨Breg细胞在IgG4-RD发病机制中的作用，本课题将研究以下内容：1）通过流式检测、ELISA等方法，对Breg细胞在IgG4-RD患者外周血中的表达和分泌IL-10的能力进行检测；2）通过检测IgG4-RD患者外周血中Breg细胞与效应T细胞共培养后T细胞的活化探讨该病中Breg细胞对效应T细胞和调节性T细胞的影响。3）CD40L刺激后Breg细胞NF-kB和AP-1信号通路的活化情况。4）受累组织中Breg细胞的表达。

IgG4 related disease (IgG4-RD) is a newly recognized systemic autoimmune disease characterized by high level of serum IgG4, massive infiltration of lymphocytes and IgG4 positive plasma cells in involved organs. Regulatory B cells (Breg)is a B cell subset which produce IL-10 and plays suppressive and regulatroy roles in immune response. According to literature, there is no study related to the role of Breg in the pathogenesis of IgG4-RD. In this study, we will investigate the following aspects of Breg in IgG4-RD: 1）Expression of Breg and it's cytokines in peripheral blood; 2) To test the suppressive/regulatory effect of Breg cells on T cells by co-culture of Breg cells and T cell; 3) NF-kB signaling pathway activation under stimulation of CD40 in Breg cells from IgG4-RD patients;4) Histopathological expression of CD24+CD38+ cells in involved organs.