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, ultimately 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 environmental 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 questions drawn from the National Health and Nutrition Examination Survey in an attempt to identify risk factors for the disease; 83.3% of the patients returned the questionnaires [3]. Surveys were also sent to siblings and friends of the cases. The female -to-male ratio among cases was 10:1, and the mean age of respondents was 53 years, accurately reflecting the general knowledge on epidemiology of PBC. The investigators identified that autoimmune diseases (Sjogren’s syndrome, Raynaud’s syndrome, autoimmune thyroid disease) were 3–15 times more frequent among PBC cases than their siblings or cases and there was a 6% prevalence of PBC in first degree relatives of cases. In addition, several illnesses were seen in association with PBC, such as urinary tract infection, tonsillectomy, cholecystectomy and shingles, raising the possibility that an infectious agent may also be involved in the pathogenesis of PBC. Symptoms begin insidiously. Fatigue is the most common symptom, present in up to 70% of patients [4], followed by pruritus, which can be quite distressing for the patient. Jaundice develops later, usually associated with progression of the disease. Other symptoms are related to portal hypertension and other sequelae of advanced cholestatic liver disease. There are several prognostic models for predicting survival, with the serum bilirubin level being the only variable common to all of them. The prognosis is noticeably different for asymptomatic or symptomatic patients, but it is clearly documented that even asymptomatic patients have a shorter survival compared to the general population [5]. The overall survival rate ranged from 5.5 to 11.9 years in the pre-transplant era [6]. A Kaplan–Meier graphic of the estimated survival in PBC is shown in Fig. 1. Therapy is aimed at preventing disease progression as well as treating or preventing its complications, namely osteoporosis, fat-soluble vitamin deficiencies, pruritus and portal hypertension.