IgG4相关性疾病(immunoglobulin G4-related disease,IgG4-RD)是一种免疫炎症介导的,以受累器官肿块形成为特征的疾病,可影响多个器官和系统,如泪腺、唾液腺、胰腺、甲状腺、前列腺、肝、肾、胃肠道、腹膜后组织和动脉、中枢神经系统等。特征性病理学表现有浆细胞炎性浸润、席纹状纤维化,静脉血管炎导致的闭塞性静脉炎、轻到中度的组织嗜酸性粒细胞增多[1]。现复习山东大学附属省立医院风湿免疫科收治的1例原发性IgG4相关性疾病患 Immunoglobulin G4-related disease (IgG4-RDD) is an immune-inflammation-mediated disease characterized by mass formation in affected organs that affects multiple organs and systems such as the lacrimal gland, salivary gland, pancreas, thyroid gland , Prostate, liver, kidney, gastrointestinal tract, retroperitoneal tissue and arteries, central nervous system and the like. Characteristic pathological manifestations of inflammatory cell infiltration of plasma cells, myxoidal sclerosis, venous vasculitis caused by occlusive phlebitis, mild to moderate tissue eosinophilia [1]. Now review of Shandong Provincial Affiliated Provincial Hospital, Department of Rheumatology, 1 case of primary IgG4-related diseases