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