Contributor: Gordon K. Klintworth
Primary biliary cirrhosis is a specific type of liver cirrhosis [cirrhosis - liver]. In this variety of cirrhosis there is a destruction of the basement membrane in the intracanalicular bile ducts of the liver. The basement membrane is well shown with the periodic acid-Schiff reaction. The serum AMA titer, alkaline phosphatase, and gamma-glutamyltransferase are elevated. p-ANCA are not present.  In primary biliary cirrhosis subcutaneous xanthomas are associated with hypercholesterolemia and an lipoprotein-X. Most of the cholesterol in biliary cirrhosis is in the high-density lipoprotein fraction and is rarely associated with atherosclerosis.