IgG4相关性硬化性胆管炎的研究现状

摘要

IgG4相关性硬化性胆管炎(IgG4-SC)是新近发现的一类发病机制不明的继发性硬化性胆管炎.以血清IgG4浓度升高、慢性进行性梗阻性黄疸、组织结构中大量IgG4阳性浆细胞和淋巴细胞弥漫或局限性浸润、纤维化和闭塞性静脉炎为特征.综述了IgG4-SC的诊断、治疗以及其与原发性硬化性胆管炎和胆管癌之间的关系.%Immunoglobulin G4-related sclerosing cholangitis (IgG4-SC) is a new type of secondary sclerosing cholangitis which was discovered recently and has unknown pathogenesis.IgG4-SC is characterized by an increased serum level of immunoglobulin G4 (IgG4),chronic progressive obstructive jaundice,diffuse or local infiltration of a large number of IgG4-positive plasma cells and lymphocytes,fibrosis,and obstructive phlebitis.This article reviews the diagnosis and treatment of IgG4-SC and its association with primary sclerosing cholangitis and cholangiocarcinoma.