Incidence and prevalence of primary biliary cirrhosis in a defined population in Sweden

The incidence and prevalence of antimitochondrial antibody-positive primary biliary cirrhosis (PBC) has been studied within a defined area in Sweden served by one hospital. During the period 1976-1983 the yearly incidence of PBC was 1.4/10(5) inhabitants, and on 31 December 1983 the prevalence was 12.8/10(5) inhabitants. The prevalence is the highest reported so far. At the time of diagnosis half of the patients were clinically asymptomatic. Two of the patients also had celiac disease with osteomalacia responding to a gluten-free diet. Gallstone disease occurred in 30% of the patients. Four patients died--two of liver-related complications, one of colonic carcinoma, and one of staphylococcal septicemia and endocarditis. One further patient, who is still alive, developed hypernephroma. Our results indicate that PBC is a fairly benign disease in most patients, with a slow progress during which they lead a fairly normal life.