Pathological Features of Autoimmune Liver Diseases / 胃肠病学

ABSTRACT

Autoimmune hepatitis (AIH),primary biliary cholangitis (PBC,formerly known as primary biliary cirrhosis),primary sclerosing cholangitis (PSC),IgG4-related sclerosing cholangitis (IgG4-SC) and IgG4-related hepatopathy are the main categories of autoimmune liver diseases (AILDs),and liver biopsy is important for their diagnosis and management. The characteristic histological features of AIH are interface hepatitis with portal lymphoplasmacytic infiltration,hepatic rosette formation and emperipolesis. Chronic nonsuppurative destructive cholangitis and epithelioid granuloma are frequently seen in PBC. PSC is characterized by 'onion-skin'type periductal fibrosis,while storiform fibrosis with IgG4-positive plasmacytic infiltration usually indicates IgG4-SC. Closer cooperation between clinicians and pathologists may improve the diagnosis and management of AILDs.