Literature DB >> 30980847

Ursodeoxycholic acid therapy and liver transplant-free survival in patients with primary biliary cholangitis.

Maren H Harms1, Henk R van Buuren1, Christophe Corpechot2, Douglas Thorburn3, Harry L A Janssen4, Keith D Lindor5, Gideon M Hirschfield6, Albert Parés7, Annarosa Floreani8, Marlyn J Mayo9, Pietro Invernizzi10, Pier Maria Battezzati11, Frederik Nevens12, Cyriel Y Ponsioen13, Andrew L Mason14, Kris V Kowdley15, Willem J Lammers1, Bettina E Hansen16, Adriaan J van der Meer17.   

Abstract

BACKGROUND & AIMS: The clinical efficacy of ursodeoxycholic acid (UDCA) in primary biliary cholangitis (PBC) remains subject to debate as definitive randomized controlled trials are lacking. We aimed to determine whether UDCA prolongs liver transplant (LT)-free survival in patients with PBC.
METHODS: This international cohort study included patients from the Global PBC Study Group database, originating from 8 countries in Europe and North America. Both UDCA-treated and untreated patients were included. LT and death were assessed as a combined endpoint through Cox regression analyses, with inverse probability treatment weighting (IPTW).
RESULTS: In the 3,902 patients included, the mean (SD) age was 54.3 (11.9) years, 3,552 patients (94.0%) were female, 3,529 patients (90.4%) were treated with UDCA and 373 patients (9.6%) were not treated. The median (interquartile range) follow-up was 7.8 (4.1-12.1) years. In total, 721 UDCA-treated patients and 145 untreated patients died or underwent LT. After IPTW, the 10-year cumulative LT-free survival was 79.7% (95% CI 78.1-81.2) among UDCA-treated patients and 60.7% (95% CI 58.2-63.4) among untreated patients (p <0.001). UDCA was associated with a statistically significant reduced risk of LT or death (hazard ratio 0.46, 95% CI 0.40-0.52; p <0.001). The hazard ratio remained statistically significant in all stages of disease. Patients classified as inadequate biochemical responders after 1 year of UDCA had a lower risk of LT or death than patients who were not treated (adjusted hazard ratio 0.56; 95% CI 0.45-0.69; p <0.001).
CONCLUSION: The use of UDCA improves LT-free survival among patients with PBC, regardless of the disease stage and the observed biochemical response. These findings support UDCA as the current universal standard of care in PBC. LAY
SUMMARY: In this international multicenter study of 3,902 patients with primary biliary cholangitis, we found that treatment with ursodeoxycholic acid is associated with prolonged liver transplant-free survival. This association was significant, irrespective of sex, age, or disease stage. The survival benefit remained statistically significant in patients with an incomplete biochemical response to ursodeoxycholic acid therapy.
Copyright © 2019 European Association for the Study of the Liver. Published by Elsevier B.V. All rights reserved.

Entities:  

Keywords:  Cholestasis; Clinical trials; Mortality; Patient management; Treatment; UDCA, transplantation

Mesh:

Substances:

Year:  2019        PMID: 30980847     DOI: 10.1016/j.jhep.2019.04.001

Source DB:  PubMed          Journal:  J Hepatol        ISSN: 0168-8278            Impact factor:   25.083


  31 in total

Review 1.  Diagnosis and treatment of primary biliary cholangitis.

Authors:  Alena Laschtowitz; Rozanne C de Veer; Adriaan J Van der Meer; Christoph Schramm
Journal:  United European Gastroenterol J       Date:  2020-04-16       Impact factor: 4.623

Review 2.  Primary biliary cholangitis: pathogenesis and therapeutic opportunities.

Authors:  Aliya F Gulamhusein; Gideon M Hirschfield
Journal:  Nat Rev Gastroenterol Hepatol       Date:  2019-12-09       Impact factor: 46.802

3.  Rates of decompensation, hepatocellular carcinoma and mortality in AMA-negative primary biliary cholangitis cirrhosis.

Authors:  Binu V John; Bassam Dahman; Yangyang Deng; Nidah S Khakoo; Tamar H Taddei; David E Kaplan; Cynthia Levy
Journal:  Liver Int       Date:  2021-10-12       Impact factor: 5.828

Review 4.  Ursodeoxycholic Acid Decreases Incidence of Primary Biliary Cholangitis and Biliary Complications After Liver Transplantation.

Authors:  Mariana C Zapata; Mark R Pedersen
Journal:  Clin Liver Dis (Hoboken)       Date:  2022-01-28

Review 5.  A Current Understanding of Bile Acids in Chronic Liver Disease.

Authors:  Naba Farooqui; Anshuman Elhence
Journal:  J Clin Exp Hepatol       Date:  2021-08-23

6.  Impact of Obeticholic acid Exposure on Decompensation and Mortality in Primary Biliary Cholangitis and Cirrhosis.

Authors:  Binu V John; Kaley Schwartz; Cynthia Levy; Bassam Dahman; Yangyang Deng; Paul Martin; Tamar H Taddei; David E Kaplan
Journal:  Hepatol Commun       Date:  2021-05-06

7.  REPLY.

Authors:  Binu V John; Yangyang Deng; Paul Martin; Cynthia Levy; Tamar H Taddei; David E Kaplan; Bassam Dahman
Journal:  Hepatology       Date:  2021-08-15       Impact factor: 17.298

8.  External Validation of UDCA Response Score in Slovak and Croatian Patients with Primary Biliary Cholangitis.

Authors:  Jakub Gazda; Martin Janicko; Sylvia Drazilova; Ivica Grgurevic; Tajana Filipec Kanizaj; Tomas Koller; Beatrica Bodorovska; Maja Mijic; Ivana Mikolasevic; Ivana Knezevic Stromar; Branislav Kucinsky; Matej Gazda; Peter Jarcuska
Journal:  Can J Gastroenterol Hepatol       Date:  2021-06-22

9.  Predictors for incomplete response to ursodeoxycholic acid in primary biliary cholangitis. Data from a national registry of liver disease.

Authors:  Helena Cortez-Pinto; Rodrigo Liberal; Susana Lopes; Mariana V Machado; Joana Carvalho; Teresa Dias; Arsénio Santos; Cláudia Agostinho; Pedro Figueiredo; Rafaela Loureiro; Alexandra Martins; Gonçalo Alexandrino; Isabel Cotrim; Carina Leal; José Presa; Mónica Mesquita; Joana Nunes; Catarina Gouveia; Ana Horta E Vale; Ana Luísa Alves; Mariana Coelho; Luís Maia; Isabel Pedroto; António Banhudo; João Sebastião Pinto; Marta Vargas Gomes; Joana Oliveira; Valeska Andreozzi; Filipe Calinas
Journal:  United European Gastroenterol J       Date:  2021-06-08       Impact factor: 4.623

Review 10.  Autoimmune biliary diseases: primary biliary cholangitis and primary sclerosing cholangitis.

Authors:  Samantha Sarcognato; Diana Sacchi; Federica Grillo; Nora Cazzagon; Luca Fabris; Massimiliano Cadamuro; Ivana Cataldo; Claudia Covelli; Alessandra Mangia; Maria Guido
Journal:  Pathologica       Date:  2021-06
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