免疫球蛋白G4相关性疾病(IgG4-RD)是一组病因不明的全身系统性自身免疫疾病,患者主要表现为血清IgG4水平升高、器官组织中大量淋巴浆细胞归巢及IgG4阳性浆细胞浸润~([1])。由于目前IgG4-RD在我国研究报道较少,为此本文就国内外IgG4-RD的最新研究进展综述如下。1 IgG4分子的理化性质和生物功能免疫球蛋白G(IgG)是由机体浆细胞合成并分泌的一组具 Immunoglobulin G4-related disease (IgG4-RD) is a group of systemic autoimmune diseases of unknown etiology. Patients mainly manifest as elevated serum IgG4, a large number of lymphoid plasma cells homing in organ tissues, and IgG4-positive plasma cell infiltration ~ ([1]). As IgG4-RD is rarely reported in our country at present, the latest research progress of IgG4-RD at home and abroad is summarized as follows. 1, the physical and chemical properties of IgG4 molecules and biological function of immunoglobulin G (IgG) is a group of plasma cells synthesized and secreted by the body