An autopsy case of primary sclerosing cholangitis with sequential histologic observations of the liver

A rare autopsy case of primary sclerosing cholangitis with sequential histologic observations of the liver is described. The patient, a 62-year-old female at the time of autopsy, presented with prolonged cholestasis of about 9 years duration. Initial (at 53 years) and second (at 59 years) liver biopsies disclosed fibrous enlargement of the portal tracts with loss of interlobular bile ducts, lymphoplasmacytic infiltration, a few epithelioid granulomas, piecemeal necrosis, atypical ductular proliferation and deposition of copper granules. Hypergammaglobulinemia with elevated IgM was also noted. These clinicopathological features resembled primary biliary cirrhosis. However, no florid duct lesions were found, and absence of antimitochondrial antibodies and cholangiographic demonstration of a beaded biliary tree favored a diagnosis of primary sclerosing cholangitis. The autopsied liver disclosed sclerosis and cholangioectases of the intra- and extrahepatic biliary tree in addition to biliary cirrhosis. The histology of the biliary tree disclosed nonspecific fibrosing inflammation in the extra- and intrahepatic biliary tree. Other autopsy findings included chronic thyroiditis, sialoadenitis and pancreatitis.