Liver and Bile

J Hepatol. 2024;81(4):679-689

Montano-Loza AJ, Lytvyak E, Hirschfield G, Hansen BE, Ebadi M, Berney T, Toso C, Magini G, Villamil A, Nevens F, Van den Ende N, Pares A, Ruiz P, Terrabuio D, Trivedi PJ, Abbas N, Donato MF, Yu L, Landis C, Dumortier J, Dyson JK, van der Meer AJ, de Veer R, Pedersen M, Mayo M, Manns MP, Taubert R, Kirchner T, Belli LS, Mazzarelli C, Stirnimann G, Floreani A, Cazzagon N, Russo FP, Burra P, Zigmound U, Houri I, Carbone M, Mulinacci G, Fagiuoli S, Pratt DS, Bonder A, Schiano TD, Haydel B, Lohse A, Schramm C, Rüther D, Casu S, Verhelst X, Beretta-Piccoli BT, Robles M, Mason AL, Corpechot C; Global PBC Study Group

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

Background and aims: 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. The authors evaluated a large, international, multicenter cohort to assess the performance of PBC scores in predicting 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 centers across Europe, North and South America. The median age at the time of rPBC was 58.0 years (interquartile range [IQR], 53.2–62.6), and 298 patients (90%) were female. 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 (IQR, 4.3–12.9) after rPBC diagnosis, 52 patients (16%) had graft loss and 103 (31%) died. After 1 year of UDCA initiation the histological stage at rPBC (hazard ratio [HR] = 3.97, 95% confidence interval [CI]: 1.36–11.55, p = 0.01), use of prednisone (HR = 3.18, 95% CI: 1.04–9.73, p = 0.04), ALP x ULN (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 observed for overall survival.

Conclusion: Patients with recurrent primary biliary cholangitis (rPBC) and disease activity, as indicated by standard PBC risk scores, have impaired outcomes, supporting efforts to treat recurrent disease in similar ways to pre-transplant PBC.

DOI:  10.1016/j.jhep.2024.05.010