| Abstract: | In an attempt to define the clinical spectrum of hepatobiliary disease associated with antimitochondrial antibody (AMA), 41 female and four male patients with AMA were investigated. On the basis of clinical criteria, 22 patients had primary biliary cirrhosis (group I), seven had extrahepatic biliary obstruction (group II) and 16 had other liver diseases including chronic active hepatitis, cryptogenic cirrhosis and minor abnormalities of liver function (group III). Elevations of serum cholesterol, alkaline phosphatase and bile acid levels separated the patients with primary biliary cirrhosis from those with miscellaneous liver disease, whereas in those with extrahepatic biliary obstruction results were intermediate. The cholestatic syndrome of primary biliary cirrhosis was further defined by a cholic to chenodoxycholic acid ratio > 1.0 which was found in 75 per cent of the patients with primary biliary cirrhosis but in none of those with miscellaneous liver disease. No significant differences in AMA titers or immunoglobulin M (IgM) levels were found between the groups, indicating that these tests are not correlated with cholestatic liver disease. Examination of liver tissue revealed features which were compatible with the clinical impression in the majority of cases. Cholelithiasis was present in 33 per cent of the patients. Common bile duct obstruction due to stones and/or stricture was found in six cases and carcinoma in one. Since four had fully developed cirrhosis at the time of presentation, extrahepatic biliary obstruction was probably a result of gallstone disease complicating AMA positive parenchymal liver disease. AMA seems to be a marker for idiopathic parenchymal liver disease, primarily in middle-aged women, but in itself gives no. further diagnostic information and does not exclude extrahepatic biliary obstruction. |
