Current management of primary biliary cirrhosis and primary sclerosing cholangitis

Primary biliary cirrhosis (PBC) is a chronic, cholestatic autoimmune liver disease characterized by inflammation and progressive destruction of interlobular bile ducts, ulti-mately leading to biliary cirrhosis. Population based studies have estimated the incidence of PBC as 19.1–251/ 1 000 000 in the general population [1,2]. The etiology of PBC is attributed to autoimmunity mainly due to the association with antimitochondrial antibodies, present in 95 % of PBC patients, but genetic and environ-mental factors are thought to contribute as well. In a large epidemiologic study conducted in the United States a survey was sent to 241 patients with PBC using standardized ques-tions drawn from the National Health and Nutrition Exam-ination Survey in an attempt to identify risk factors for the disease; 83.3 % of the patients returned the questionnaire