Atypical biliary cirrhosis--or sclerosing cholangitis.

Four patients had a characteristic cholestatic syndrome intermediate between primary biliary cirrhosis (PBC) and sclerosing cholangitis (SC). These patients with atypical biliary cirrhosis (ABC) differ from patients with PBC in that they do not have either antimitochondrial antibodies or elevated IgM, are younger, and three of the four are men. Patients with ABC differ from those with SC in that the common duct, hepatic duct, the right and left hepatic ducts and their immediate tributaries show none of the changes characteristic and diagnostic of SC. The findings in ABC which are similar to PBC are: 1) the earliest biopsy finding of the disease is confined to the interlobular duct and is indistinguishable from PBC; 2) late changes on cholangiogram are similar to those in a proportion of patients with PBC; and 3) the incidence of gallstones is increased. ABC has no similarity to SC except that the small intrahepatic ducts are affected by both diseases. The histologic features in fully established ABC suggest extrahepatic bile duct obstruction because of the predominantly PMN leukocytes portal inflammation. The cholangiograms show no evidence of bile duct obstruction, but there is rapid attenuation and narrowing of the smaller intrahepatic bile duct. Cholecystectomy does not improve the clinical course of ABC.