Four cases of autoimmune cholangitis

Autoimmune cholangitis is a disease with clinicopathologic features of primary biliary cirrhosis without antimitochondrial antibodies and, frequently, with high antinuclear antibody titers. Alternative terms have been suggested, including antimitochondrial antibody-negative primary biliary cirrhosis, immunocholangitis, autoimmune cholangiopathy, and autoimmune hepatobiliary overlap syndrome type 1. The question of whether autoimmune cholangitis is distinct from primary biliary cirrhosis and autoimmune hepatitis or is an antimitochondrial antibody-negative variant of primary biliary cirrhosis and autoimmune hepatitis is controversial. This case report describes the clinical, immunohistochemical, and pathological findings in four patients with autoimmune cholangitis and reviews the literature.