Recently, animal models have been discovered that may provide a new insight into the pathogenesis of this disease and facilitate appreciation for novel treatment in PBC.

Liver transplantation is usually an option for patients with liver failure and the outcome is 70% survival at 7 years. Patients, particularly those who start UDCA treatment at early-stage disease and who respond in terms of improvement of the liver biochemistry, have a good prognosis. Ursodeoxycholic acid (UDCA) is the only currently known medication that can slow the disease progression. All AMA negative patients with cholestatic liver disease should be carefully evaluated with cholangiography and liver biopsy. Diagnosis is based on a combination of clinical features, abnormal liver biochemical pattern in a cholestatic picture persisting for more than six months and presence of detectable antimitochondrial antibodies (AMA) in serum.

Environmental factors may have potential causative role (infection, chemicals, smoking). Genetic susceptibility as a predisposing factor for PBC has been suggested. PBC is associated with other autoimmune diseases such as Sjogren's syndrome, scleroderma, Raynaud's phenomenon and CREST syndrome and is regarded as an organ specific autoimmune disease. Patients may even present with ascites, hepatic encephalopathy and/or esophageal variceal hemorrhage. The majority of patients are asymptomatic at diagnosis, however, some patients present with symptoms of fatigue and/or pruritus. Annual incidence is estimated between 0.7 and 49 cases per million-population and prevalence between 6.7 and 940 cases per million-population (depending on age and sex). Affected individuals are usually in their fifth to seventh decades of life at time of diagnosis, and 90% are women. Primary biliary cirrhosis (PBC) is a chronic and slowly progressive cholestatic liver disease of autoimmune etiology characterized by injury of the intrahepatic bile ducts that may eventually lead to liver failure.

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