Primary biliary cirrhosis: survival of a cohort followed for 10 years

Objectives. To study the natural course of primary biliary cirrhosis (PBC) in order to be able to design accurate clinical pharmacological studies and evaluate the need for liver transplantation. Design. A cohort of 86 patients with PBC living in northern Sweden was foll owed for a 10-year period during 1983-93. No patients received therapy with ursodeoxy cholic acid or other drugs during the follow-up period. Method At start all patients were investigated personally by the authors. A t follow-up medical notes were scrutinized and special questionnaires to th e current responsible physician were applied. End-points were the time of d ropout, liver transplantation, death or end of the study period. Results. At follow-up data were available for 84 patients (97%). During the study period 34 patients died, of whom 28 were symptomatic; 15 deaths had no direct connection to PBC. Nineteen deaths were related to PBC of whom tw o were asymptomatic, the most common cause being end-stage liver disease wi th liver coma. During the study period in all eight patients were subjected to liver transplantation. Conclusions. The survival rate of the 32 asymptomatic PBC patients at the s tart of the study was the same as a sex- and age-matched standard backgroun d population. Those patients with symptomatic PBC from the beginning of stu dy had a survival rate at 10 years of 50%, and the most ominous sign was a bilirubin greater than 35 mu mol L-1. Liver transplantation was performed i n almost 10% in this cohort until 1993. Since then, the indications and ref erral practice for liver transplantation has changed and is now higher.