PRIMARY BILIARY-CIRRHOSIS

Primary biliary cirrhosis is a disease, of intrahepatic bile ducts. Small intrahepatic bile ducts (about 80 mum in diameter) arc infiltrated with CD4 and CD8 lymphocytes and progressively destroyed. Biliary obstruction leads to fibrosis and biliary cirrhosis in 10 to 15 years. The annual incidence is 3-19 cases per 10(6), with a strong female predominance. There is a weak association with class II HLA antigens. The most frequent symptoms are pruritus, jaundice and asthenia. Extrahepatic manifestations include Sjogren syndrome, rheumatoid arthritis, scleroderma and other autoimmune diseases. Autoantibodies against mitochondrial antigens are present in more than 90 p.100 of cases. The main antigens recognized are components of the pyruvate dehydrogenase enzyme complex, mostly the E2 subunit (dihydrolipoamide acyl transferase). The significance of these antibodies is unclear. Ursodeoxycholic acid (ursodiol) has been shown to improve pruritus, biochemical abnormalities, and, at histology, inflammation and cholestasis, but not fibrosis. It may decrease the progression of the disease. Liver transplantation should be considered when serum bilirubin is above 150 mumol/l. Survival after transplantation in such cases is considerably improved, with a 5-year survival rate increased from about 10 % to nearly 70 %.