IgG4相关硬化性胆管炎的研究进展

免疫球蛋白G4相关硬化性胆管炎(immunoglob-ulin G4-related sclerosing cholangitis,IgG4-SC)是一种新近认识的以血清IgG4升高、慢性进行性阻塞性黄疸、弥漫性或局限性IgG4阳性浆细胞和淋巴细胞组织浸润、纤维化及闭塞性静脉炎为特征的慢性炎症性疾病,常并发自身免疫性胰腺炎(autoimmune pancreatitis,AIP),其临床、生化及影像学特征与原发性硬化性胆管炎(primary sclerosing cholangitis,PSC)或胆管癌(cholangiocarcinoma,CC)相似.类固醇激素是IgG4-SC的主要治疗手段,而肝移植是PSC唯一的有效治疗方法,CC则需外科手术治疗.因此,IgG4-SC与PSC或CC间的准确鉴别是目前面临的一个十分重要的课题.本文详尽地阐述了免疫球蛋白G4(immunoglobulin G4,IgG4)的特征和功能,IgG4-SC的诊断和治疗,IgG4-SC与AIP、PSC及CC之间关系等研究进展,为IgG4-SC的精确诊断和治疗提供了新的思路.

Recent advances in research of immunoglobulin G4-related sclerosing cholangitis

Immunoglobulin G4-related sclerosing cholangitis(IgG4-SC) is a recently def ined disease entity characterized by elevated serum IgG4,chronic progressive obstructive jaundice,and diffuse or mass-forming inflammatory reaction rich in IgG4-positive plasma cells and lymphocytes associated with fibrosclerosis and obliterative phlebitis,which shares a number of clinical,biochemical,and radiological features with primary sclerosing cholangitis(PSC) or cholangiocarcinoma(CC).IgG4-SC is commonly associated with autoimmune pancreatitis(AIP).Steroid therapy comprises the mainstay of treatment for IgG4SC patients.However,liver transplantation is the only useful treatment for PSC patients,and CC patients require surgical therapy.Therefore,the accurate discrimination between IgG4-SC and PSC or CC is a very important issue.In this article,we will review the features and role of immunoglobulin G4(IgG4),the diagnosis andtherapy of IgG4-SC,and the relations between IgG4-SC and AIP,PSC or CC.