Clinical epidemiology of primary biliary cirrhosis: incidence, prevalence, and impact of therapy.

Primary biliary cirrhosis (PBC) is a chronic cholestatic liver disease of unknown etiology. It is typically characterized by a trio of criteria including cholestatic liver biochemistry tests for at least 6 month's duration, the presence of a positive serum antimitochondrial antibody, and histologic findings from liver biopsy that are compatible or diagnostic with PBC. Although no unifying mechanism of action has been described to explain the pathogenesis of PBC to date, the natural history of this condition is greatly influenced by several known and unknown risk factors. In turn, a number of publications have focused on describing the burden of disease and prognosis in patients with PBC, This review article will summarize the descriptive epidemiology of PBC including new insights on risk factors, prognosis, and the effect of medical therapy in contemporary patients.