Primary intrahepatic obliterating cholangitis: a possible variant of 'sclerosing cholangitis'

A sclerosing cholangitis confined to the intrahepatic ducts occurred in a 64-year-old man with a clinical picture of prolonged cholestatic jaundice. Two laparotomies and an operative cholangiogram demonstrated a normal extrahepatic biliary tree. The most prominent histological feature in the liver biopsy was the replacement of medium-sized bile ducts by fibrous cords. Electron microscopic examination of the liver showed changes attributable to cholestasis and cholesterol clefts in dense bodies. Needle-like and curved membrane-bounded inclusions of an obscure nature were also observed in some of the dense bodies. It is suggested that this case, together with nine histologically similar cases which have been labelled in the literature as ;primary biliary cirrhosis', represents a subgroup of sclerosing cholangitis in which only the intrahepatic ducts are affected.