[Primary biliary cirrhosis]

Primary biliary cirrhosis is a chronic cholestatic disease which usually affects middle-aged women and is characterized by portal vein inflammation and by segmental and focal necrosis of small intrahepatic bile ducts. The prevalence of the disease is estimated at 8 to 12 cases for 100,000 inhabitants. Genetic, infectious and/or immunological factors acting together may be responsible for small bile duct destruction. The main consequence of this destruction is cholestasis. As in all types of mechanical cholestasis, so-called lobular lesions such a fibrosis or even cirrhosis may then develop. Clinically, primary biliary cirrhosis evolves in three phases: (1) a preclinical asymptomatic phase where the disease is revealed by the accidental discovery of antimitochondrial antibodies or of a moderate rise in gammaglutamyltranspeptidase or serum alkaline phosphatase activity; (2) a clinical phase, usually lasting 5 to 10 years, characterized by fatigue, pruritus and later, clinical signs directly related to the hepatic lesions; (3) a terminal phase marked by major cholestasis with lesions of fibrosis or cirrhosis and sometimes ascites and portal hypertension responsible for gastrointestinal haemorrhages. In the last few years the prognosis of primary biliary cirrhosis has been considerably improved by the introduction and development of liver transplantation (the first choice treatment in the terminal phase) and by the introduction of ursodeoxycholic acid.