Primary biliary cirrhosis (PBC) is a chronic, cholestatic liver disease which affects mainly middle-aged women. It starts with an inflammatory process of the small and middle-sized interlobular bile ducts leading first to a proliferation and then to a loss of bile ducts, to portal inflammation and in late stages to liver cirrhosis.
PBC occurs all over the world but with varying incidence, ranging from 0.7-49 per million per year. In most recent studies the point prevalence was estimated to range from 6.7 to 402 per million.
Typical clinical features of PBC are fatigue, pruritus and Sicca-syndrome. However, nowadays at diagnosis, the majority of patients are asymptomatic and present for other reasons, e.g. for workup of elevated serum levels of AP or cholesterol. A diagnosis of PBC is made “with confidence” when biochemical markers of cholestasis, particularly alkaline phosphatase, are elevated persistently for more than 6 months in the presence of antibodies against mitochondria and in the absence of an alternative explanation.
PBC-related antibodies against mitochondria react with subunits of the 2-oxoaciddehydrogenase complex (2-OADC) and, in most cases, recognize the E2-subunit of pyruvate dehydrogenase (PDH-E2). Individuals, who are positive for these so-called antiM2 antibodies, even if they have no signs of cholestasis and/or liver inflammation, are very likely to develop PBC.
Anti-M2 antibodies are present in about 95% of PBC-patients.
Preparation of Patient: No special preparation.
