Coexistence of autoimmune pancreatitis and primary biliary cirrhosis in a Caucasian patient - a rare cause of cholestasis.

In contrast to Asia, autoimmune pancreatitis is rare in the Western population. The present case report describes the coexistence of primary biliary cirrhosis and autoimmune pancreatitis accompanied by sclerosing cholangitis of the intrahepatic bile ducts and the hilar region. It is important to differentiate sclerosing cholangitis due to autoimmune pancreatitis from primary sclerosing cholangitis and primary biliary cirrhosis, because the former responds to steroids, while the latter do not. The article highlights important diagnostic difficulties and suggests racial differences between Caucasian and Asian patients.