Intrahepatic cholangiographic appearance simulating primary sclerosing cholangitis in several hepatobiliary diseases: a postmortem cholangiographic and histopathological study in 154 livers at autopsy.

Intrahepatic cholangiography of primary sclerosing cholangitis (PSC) is characterized by stricture with or without dilation of the biliary tree. To evaluate whether this cholangiographic appearance is present in non-PSC livers as well as the histological features seen in non-PSC livers with this cholangiographic appearance, we performed postmortem intrahepatic cholangiography in 154 liver autopsy specimens. The PSC-like cholangiographic appearance was frequently found in cirrhosis with or without hepatocellular carcinoma (4 of 6, 67%), hepatocellular carcinoma (1 of 1, 100%), adult-type polycystic disease of the liver and kidneys (2 of 3, 67%), submassive hepatic necrosis (2 of 5, 40%), amyloidosis (1 of 2, 50%), and intrahepatic extensive thrombosis (1 of 1, 100%). It was also found but at lower frequency in metastatic carcinomas (3 of 13, 23%) and leukemia/lymphoma infiltration (2 of 12, 17%). Histologically, in livers with such a PSC-like cholangiographic appearance, the intrahepatic bile ducts were compressed by fibrosis, inflammatory infiltrates, liver cysts, cancer cell infiltration, amyloid deposition, or portal thrombi. Dilated ducts had less pronounced changes than strictured ducts. In these hepatobiliary diseases, the changes of intrahepatic bile ducts in the livers without the PSC-like cholangiographic appearance were much less marked than those in the livers with it. These data suggest that the PSC-like intrahepatic cholangiographic appearance is present in several hepatobiliary diseases and that clinicians should take such diseases into consideration if stricture with or without dilation is found on intrahepatic cholangiography.