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; ... (2024). Prognostic Scores for Ursodeoxycholic Acid-Treated Patients Predict Graft Loss and Mortality in Recurrent Primary Biliary Cholangitis after Liver Transplantation. Journal of hepatology, 81(4), pp. 679-689. Elsevier 10.1016/j.jhep.2024.05.010
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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 [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 (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 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 pre-transplant PBC.
IMPACT AND IMPLICATIONS
One in three people who have 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 emphasized supporting efforts to treat recurrent disease in similar ways to pre-transplant primary biliary cholangitis.
Item Type: |
Journal Article (Original Article) |
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Division/Institute: |
04 Faculty of Medicine > Department of Gastro-intestinal, Liver and Lung Disorders (DMLL) > Clinic of Visceral Surgery and Medicine > Hepatology 04 Faculty of Medicine > Department of Gastro-intestinal, Liver and Lung Disorders (DMLL) > Clinic of Visceral Surgery and Medicine |
UniBE Contributor: |
Stirnimann, Guido, Casu, Stefania |
Subjects: |
600 Technology > 610 Medicine & health |
ISSN: |
0168-8278 |
Publisher: |
Elsevier |
Language: |
English |
Submitter: |
Pubmed Import |
Date Deposited: |
03 Jun 2024 14:28 |
Last Modified: |
20 Sep 2024 00:13 |
Publisher DOI: |
10.1016/j.jhep.2024.05.010 |
PubMed ID: |
38821360 |
Uncontrolled Keywords: |
autoimmune liver disease graft survival liver transplantation recurrent disease survival |
BORIS DOI: |
10.48350/197440 |
URI: |
https://boris.unibe.ch/id/eprint/197440 |