PRIMARY BILIARY-CIRRHOSIS IN ESTONIA - WITH SPECIAL REFERENCE TO INCIDENCE, PREVALENCE, CLINICAL-FEATURES, AND OUTCOME

Background: Primary biliary cirrhosis (PBC) is a liver disease of unkn own etiology, whose occurrence varies greatly between different region s. For a long time there have been no published data about the inciden ce and prevalence of PBC from Eastern Europe countries. Methods: The i ncidence and prevalence of PBC have been investigated in the Estonian population during the period 1973-92. Two sources of information were used: an information circular/questionnaire was sent to all district h ospitals and gastroenterologists, and the case histories of all patien ts with a positive antimitochondrial antibody titer of 1:40 or more we re reexamined. Results: During this period 69 cases of PBC were diagno sed. The male to female ratio was 1:22; 13% of the patients were asymp tomatic. The mean annual incidence was 2.27 per million, and on 31 Dec ember 1992 the point prevalence was 26.9 per million. There were diffe rences in prevalence among the various districts of Estonia. Associate d autoimmune conditions were reported in 32% of the patients. Mean sur vival from the time of diagnosis was 52.5 months. Conclusions: The inc idence of PBC in Estonia is at the lower end of the range reported in the world literature. This has probably partly been caused by a low pe rcentage of asymptomatic and male patients.