RESUMO
Immunoglobulin G4-related disease (IgG4-RD)is an immune-mediate disease with common specific pathology,serological,and clinical features. IgG4-related autoimmune pancreatitis,hepatopathy and sclerosing cholangitis have characteristics of the focal or diffuse swelling of affected organs and tissues,elevated serum concentration of IgG4, rich IgG4 positive lymphoplasmacytic infiltration,different degrees of storiform pattern of fibrosis and obliterative phlebitis. They also have special characteristics in clinical manifestations,diagnosis and treatment. IgG4-RD generally responds to glucocorticoid with excellent prognosis,but is easily recurrent. This article summarized the advances in pathogenesis, diagnosis and treatment of IgG4-related autoimmune pancreatitis,hepatopathy and sclerosing cholangitis.