PRIMARY BILIARY-CIRRHOSIS

Primary biliary cirrhosis was described in detail by POPPER et al. in 1965. Segmentary necrosis of the interlobular bile ducts leads to progressive cholestasis of the mechanical type. Three stages, asymptomatic, cholestatic and finally terminal, are well known. The disease appears to be induced by genetic as well as by infectious and immunologic factors. There is a higher incidence of some HLA groups and sulphoxidation is deficient. Viruses such as HCV and CMV have been shown to be capable of damaging bile duct epithelia. The group most prone to develop the disease are women aged 30-65 years. In 30-40% diagnosis is already possible in the asymptomatic stage. Three criteria are of importance: cholestasis, elevated IgM and/or antimitochondrial antibodies, and histologic evidence of chronic aggressive cholangitis. Antimitochondrial antibodies of the M2 and M9 type are characteristic features. Their titer must be above 1:100. The transaminases are only slightly pathologic. Signs of hepatocellular insufficiency or of portal hypertension occur only in the advanced stages of the disease. 2-5% of patients display autoimmune symptoms such as Sjogren syndrome, Raynaud phenomenon and Crest syndrome. Steatorrhea, osteoporosis, infections of the urogenital tract and some carcinomas, such as liver and breast cancer, are known complications. The most specific feature differentiating primary biliary cirrhosis from other causes of intrahepatic cholestasis is demonstration of antimitochondrial antibodies and the typical histology. Various therapies have been evaluated in double-blind studies. Fairly good results have been achieved with cyclosporin A, though side effects have been marked and treatment with cyclosporin is therefore not recommended. High doses of the water soluble ursodeoxycholic acid replace part of the endogenous bile salts in the bile and the blood, and this may reduce cholestasis and hepatocellular toxicity. Itching disappears after 2-3 months and final decompensation of the liver appears to be reduced. The therapy of choice for terminal disease is liver transplant. The chances of survival 5 years after liver transplant for primary biliary cirrhosis are excellent.