[Primary biliary cirrhosis (PBC)].

Primary biliary cirrhosis (PBC) is an inflammatory autoimmune liver disease leading, if untreated, to cholestasis and cirrhosis. The diagnosis is made on the basis of elevated plasma levels of alkaline phosphatase and antimitochondrial antibodies at a stage where the patient usually still remains symptomless. The degree of severity and prognosis of the disease are defined by means of histological findings of the liver. Adequate response for approximately half of the patients is obtained with urodeoxycholic acid treatment. The treatment for advanced disease is hepatic transplantation, which yields excellent results.