Autoimmune cholangitis: not just AMA-negative primary biliary cirrhosis

Variant forms of chronic cholestatic liver disease in adults, such as autoimmune cholangitis, primary biliary cirrhosis-autoimmune hepatitis overlap, and primary sclerosing cholangitis-autoimmune hepatitis overlap constitute approximately 20% of cases of autoimmune liver disease. Retrospective study design may obscure subtle differences in clinical features. Careful prospective studies are needed to help clarify classification of these disorders. Autoimmune cholangitis is often considered synonymous with antimitochondrial antibody-negative primary biliary cirrhosis, but may instead be a heterogeneous group of disorders including some cases of isolated small duct primary sclerosing cholangitis. The variable response of variant syndromes to established therapies such as corticosteroids provides justification for continued efforts to define their clinicopathologic features.