Prognostic scores for ursodeoxycholic acid-treated patients predict graft loss and mortality in recurrent primary biliary cholangitis after liver transplantation

Background/aim: Recurrent primary biliary cholangitis (rPBC) develops in approximately 30% of patients and negatively impacts graft and overall patient survival after liver transplantation (LT).There is a lack of data regarding the response rate to ursodeoxycholic acid (UDCA) in rPBC.We evaluated a large, international, multi-center cohort to assess the performance of scores for PBC to predict the risk of graft and overall survival after LT in patients with rPBC.Methods: A total of 332 patients with rPBC after LT were evaluated from 28 centres across Europe, North and South America.The median age at the time of rPBC was 58.0 years [IQR 53.2 -62.6], and 298 patients (90%) were females.The biochemical response was measured with serum levels of alkaline phosphatase (ALP) and bilirubin, and Paris-2, GLOBE and UK-PBC scores at 1 year after UDCA initiation.Results: During a median follow-up of 8.7 years .9]after rPBC diagnosis, 52 patients (16%) had graft loss and 103 (31%) died.After 1 year of UDCA initiation the histological stage at rPBC (HR, 3.97, P=0.01), use of prednisone (HR 3.18, 95%CI 1.04-9.73,P=0.04), ALP xULN (HR 1.59, 95%CI 1.26-2.01,P<0.001), Paris-2 criteria (HR 4.14, 95%CI 1.57-10.92,P=0.004), GLOBE score (HR 2.82, 95%CI 1.71-4.66,P<0.001), and the UK-PBC score (HR 1.06, 95%CI 1.03-1.09,P<0.001) were associated with graft survival in the multivariate analysis.Similar results were found in the overall survival analysis. Conclusion:Patients with rPBC and disease activity as indicated by standard PBC risk scores have impaired outcomes, supporting efforts to treat recurrent disease in similar ways to pretransplant PBC.