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Lookup NU author(s): Dr Jess Dyson
This work is licensed under a Creative Commons Attribution 4.0 International License (CC BY 4.0).
© 2024 The AuthorsBackground & 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. We evaluated a large, international, multi-center 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 [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% CI 1.36-11.55, 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 observed for overall survival. 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 pre-transplant PBC. Impact and implications: One in three people who undergo liver transplantation for primary biliary cholangitis develop recurrent disease in their new liver. Patients with recurrent primary biliary cholangitis and incomplete response to ursodeoxycholic acid, according to conventional prognostic scores, have worse clinical outcomes, with higher risk of graft loss and mortality in similar ways to the disease before liver transplantation. Our results supportsupport efforts to treat recurrent disease in similar ways to pre-transplant primary biliary cholangitis.
Author(s): 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, Ruther D, Casu S, Verhelst X, Beretta-Piccoli BT, Robles M, Mason AL, Corpechot C
Publication type: Article
Publication status: Published
Journal: Journal of Hepatology
Year: 2024
Pages: epub ahead of print
Online publication date: 29/05/2024
Acceptance date: 07/05/2024
Date deposited: 15/07/2024
ISSN (print): 0168-8278
ISSN (electronic): 1600-0641
Publisher: Elsevier B.V.
URL: https://doi.org/10.1016/j.jhep.2024.05.010
DOI: 10.1016/j.jhep.2024.05.010
Data Access Statement: The datasets generated and analysed during the current study are not publicly available but are available from the corresponding author at reasonable request.
PubMed id: 38821360
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