Prognostic Scores for Ursodeoxycholic Acid-Treated Patients Predict Graft Loss and Mortality in Recurrent Primary Biliary Cholangitis after Liver Transplantation
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Prognostic Scores for Ursodeoxycholic Acid-Treated Patients Predict Graft Loss and Mortality in Recurrent Primary Biliary Cholangitis after Liver Transplantation. / Montano-Loza, Aldo J; Lytvyak, Ellina; Hirschfield, Gideon; Hansen, Bettina E; Ebadi, Maryam; Berney, Thierry; Toso, Christian; Magini, Giulia; Villamil, Alejandra; Nevens, Frederik; Van den Ende, Natalie; Pares, Albert; Ruiz, Pablo; Terrabuio, Débora; Trivedi, Palak J; Abbas, Nadir; Donato, Maria Francesca; Yu, Lei; Landis, Charles; Dumortier, Jérôme; Dyson, Jessica Katharine; van der Meer, Adriaan J; de Veer, Rozanne; Pedersen, Mark; Mayo, Marlyn; Manns, Michael P; Taubert, Richard; Theresa, Kirchner; Belli, Luca S; Mazzarelli, Chiara; Stirnimann, Guido; Floreani, Annarosa; Cazzagon, Nora; Russo, Francesco Paolo; Burra, Patrizia; Zigmound, Udi; Houri, Inbal; Carbone, Marco; Mulinacci, Giacomo; Fagiuoli, Stefano; Pratt, Daniel Stephan; Bonder, Alan; Schiano, Thomas D; Haydel, Brandy; Lohse, Ansgar; Schramm, Christoph; Rüther, Darius; Casu, Stefania; Verhelst, Xavier; Beretta-Piccoli, Benedetta Terziroli; Robles, Mercedes; Mason, Andrew L; Corpechot, Christophe; Global PBC Study Group.
In: J HEPATOL, Vol. 81, No. 4, 10.2024, p. 679-689.Research output: SCORING: Contribution to journal › SCORING: Journal article › Research › peer-review
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TY - JOUR
T1 - Prognostic Scores for Ursodeoxycholic Acid-Treated Patients Predict Graft Loss and Mortality in Recurrent Primary Biliary Cholangitis after Liver Transplantation
AU - Montano-Loza, Aldo J
AU - Lytvyak, Ellina
AU - Hirschfield, Gideon
AU - Hansen, Bettina E
AU - Ebadi, Maryam
AU - Berney, Thierry
AU - Toso, Christian
AU - Magini, Giulia
AU - Villamil, Alejandra
AU - Nevens, Frederik
AU - Van den Ende, Natalie
AU - Pares, Albert
AU - Ruiz, Pablo
AU - Terrabuio, Débora
AU - Trivedi, Palak J
AU - Abbas, Nadir
AU - Donato, Maria Francesca
AU - Yu, Lei
AU - Landis, Charles
AU - Dumortier, Jérôme
AU - Dyson, Jessica Katharine
AU - van der Meer, Adriaan J
AU - de Veer, Rozanne
AU - Pedersen, Mark
AU - Mayo, Marlyn
AU - Manns, Michael P
AU - Taubert, Richard
AU - Theresa, Kirchner
AU - Belli, Luca S
AU - Mazzarelli, Chiara
AU - Stirnimann, Guido
AU - Floreani, Annarosa
AU - Cazzagon, Nora
AU - Russo, Francesco Paolo
AU - Burra, Patrizia
AU - Zigmound, Udi
AU - Houri, Inbal
AU - Carbone, Marco
AU - Mulinacci, Giacomo
AU - Fagiuoli, Stefano
AU - Pratt, Daniel Stephan
AU - Bonder, Alan
AU - Schiano, Thomas D
AU - Haydel, Brandy
AU - Lohse, Ansgar
AU - Schramm, Christoph
AU - Rüther, Darius
AU - Casu, Stefania
AU - Verhelst, Xavier
AU - Beretta-Piccoli, Benedetta Terziroli
AU - Robles, Mercedes
AU - Mason, Andrew L
AU - Corpechot, Christophe
AU - Global PBC Study Group
N1 - Copyright © 2024. Published by Elsevier B.V.
PY - 2024/10
Y1 - 2024/10
N2 - BACKGROUND & 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.
AB - BACKGROUND & 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.
U2 - 10.1016/j.jhep.2024.05.010
DO - 10.1016/j.jhep.2024.05.010
M3 - SCORING: Journal article
C2 - 38821360
VL - 81
SP - 679
EP - 689
JO - J HEPATOL
JF - J HEPATOL
SN - 0168-8278
IS - 4
ER -
