Background/Aims: Liver transplantation (LTx) is the only established treatm
ent in patients with end-stage primary biliary cirrhosis (PBC). Although sh
ort-term survival after LTx in this group of patients is usually good, few
data exist on the long-term survival. The optimal timing of transplantation
is difficult. Thus, the aims of this study were to assess the long-term su
rvival of patients with PBC after LTx and to identify potential predictive
factors for a positive outcome. Methods: Survival of 28 patients with PBC w
ho underwent LTx between 1985 and July 1999 in a single center was studied
by Kaplan-Meier analysis and was compared to predicted survival without LTx
using established prognostic models for PBC, the Mayo and European risk sc
ores. Potential prognostic parameters obtained before LTx were tested for c
orrelation to survival. Rates of bone fractures as markers of hepatic osteo
dystrophy were compared before and after LTx. Results: Median follow-up aft
er LTx was 90 months with a maximum of 140 months. Actuarial survival of pa
tients with PBC was 89% after 1, 5, and 10 years and was significantly bett
er than estimated survival without LTx after 1-7 years as calculated by the
Mayo and European risk scores. Of several parameters tested, only serum bi
lirubin and the prognostic scores, but no other liver function tests obtain
ed immediately prior to transplantation were significantly correlated with
survival after LTx. The duration of intensive care after LTx was not associ
ated with any parameters obtained before LTx. Bone fractures were diagnosed
in 43% of patients of whom the vast majority were osteopenic before LTx as
determined by osteodensitometry. Conclusion: Longterm survival of a well-d
efined group of patients with PBC was excellent after LTx and was inversely
correlated with preoperative serum bilirubin levels as well as Mayo and Eu
ropean risk scores. Copyright (C) 2000 S. Karger AG. Basel.