Research advances in immunoglobulin G4-related sclerosing cholangitis / 临床肝胆病杂志

ABSTRACT

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.