ABSTRACT
PBC is an autoimmune disease of unknown cause, characterized by the progressive destruction of small interlobular bile ducts by a chronic granulomatous inflammation, which results in cirrhosis. The prevalence varies widely between and within different countries (between 20 and 240 cases per 1 000 000 population). The incidence appears to be increasing, particularly in north-east England. It may present in any age (except childhood), and there is a family predisposition.
