Autoimmune Diseases of the Biliary Tract: A Review

The management of autoimmune hepatobiliary disorders remains a challenging and emerging area of investigation. An awareness of cholestatic liver diseases is critical to appropriate recognition and management of these challenging diseases, because patients often present asymptomatically, and diagnosis is limited by the lack of disease-specific markers and diagnostic studies. Furthermore, there is a paucity of treatment options because the pathophysiology underlying autoimmune biliary diseases remains largely unknown. This article discusses the natural history, clinical presentation, diagnosis, and medical and surgical management strategies for three dominant autoimmune biliary diseases: primary biliary cirrhosis, primary sclerosing cholangitis, and immunoglobulin G4-related hepatobiliary disease.