An autopsy case of primary biliary cirrhosis associated with hepatic nodular hyperplasia

Abstract

We report an autopsy case of primary biliary cirrhosis (PBC) associated with hepatic nodular hyperplasia which spread extensively throughout the liver. The patient was 50-year-old female who was diagnosed as having PBC based on the liver biopsy and the demonstration of serum mitochondrial antibody, and died of respiratory failure of unknown etiology. The liver weighting 730g was atrophic, but lacked a macroscopic feature of iver cirrhosis. The cut surface showed numerous whitish nodules consisting of the hyperplastic hepatocytes. Autopsy also revealed slightly developed esophageal varices and splenomegaly, and a histological examination of the liver revealed narrowing and occlusion of the peripheral portal vein. These findings suggested that circular disturbance of the intra-hepatic portal vein which might cause portal hypertension played a role in the formation of hepatic nodules. No autopsy case of PBC with hepatic nodular hyperplasia has been reported, while Nakanuma reported nodular hyperplasia in 9 of 26 cases of early PBC through the histological examination of wedge liver biopsy.