Abstract
This paper presents an autopsy case of lupoid hepatitis with intrahepatic portosystemic shunt. The patient was an 85-year-old woman without a history of blood transfusion or trauma. She was diagnosed as having an autoimmune hepatitis 5 years before, but steroid therapy was not administrated. On suspicion of liver cirrhosis, she was admitted for further examination. Anti-nuclear antibody, anti-DNA antibody and LE cell phenomenon were all positive. Abdominal ultrasonography revealed multicystic lesion in the left lobe. An afferent vessel from the left branch of portal vein, an efferent vessel into the left hepatic vein were confirmed. Blood flow within the lesion was also confirmed by pulsed Doppler flowmetry. On the basis of these findings, intrahepatic portosystemic shunt was diagnosed. Autopsy disclosed the presence of muscle layer and elastic fiber layer at the site of the shunt. Endothelial cells were also confirmed. Surrounding vessels had no evidence of thrombus or scar formation, but macronodular cirrhosis due to lupoid hepatitis was found in the hepatic parenchyma. By histopathological evaluation of the shunt, the condition was considered as congenital. But from the clinical course, the likelihood of this being an acquired change could not be denied.
