Primary biliary cirrhosis: clinicopathological characteristics and outcome

Thirty-five patients with primary biliary cirrhosis were seen between 1974 and 1989. The median mean age at presentation was 53 years (range: 30-77) and the female to male ratio was 7.8:1. Thirteen (37%) were asymptomatic and nine (26%) had associated auto-immune disorders. Pruritus and hepatomegaly were present in half of the patients. Advanced histological stages of disease (Stages 3 and 4) were present in 57% of patients. The median period of follow-up was 5 years (range: 0.1-20). Twelve patients died, nine from hepatic causes and three from non-hepatic causes. One has undergone liver transplantation. A Kaplan-Meier curve estimated the median survival to be 11 years. Asymptomatic patients developed progressive disease and survival was similar to that of symptomatic patients. Using Cox's proportional hazards model, age, serum bilirubin and serum albumin were found to be independent prognostic variables correlating with reduced survival.