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

J Intern Med. 2000 Oct;248(4):292-8. doi: 10.1046/j.1365-2796.2000.00733.x.


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 followed 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. At follow-up medical notes were scrutinized and special questionnaires to the current responsible physician were applied. Endpoints were the time of dropout, 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 two were asymptomatic, the most common cause being end-stage liver disease with 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 start of the study was the same as a sex- and age-matched standard background population. Those patients with symptomatic PBC from the beginning of study had a survival rate at 10 years of 50%, and the most ominous sign was a bilirubin greater than 35 micromol L(-1) . Liver transplantation was performed in almost 10% in this cohort until 1993. Since then, the indications and referral practice for liver transplantation has changed and is now higher.

Publication types

  • Research Support, Non-U.S. Gov't

MeSH terms

  • Adult
  • Aged
  • Aged, 80 and over
  • Bilirubin / blood
  • Cause of Death
  • Disease Progression
  • Female
  • Follow-Up Studies
  • Humans
  • Liver Cirrhosis, Biliary / blood
  • Liver Cirrhosis, Biliary / complications
  • Liver Cirrhosis, Biliary / mortality*
  • Liver Cirrhosis, Biliary / surgery*
  • Liver Failure / etiology
  • Liver Transplantation
  • Male
  • Middle Aged
  • Needs Assessment
  • Patient Selection
  • Referral and Consultation
  • Surveys and Questionnaires
  • Survival Analysis
  • Sweden / epidemiology


  • Bilirubin