Geographical region and clinical outcomes of patients with primary biliary cholangitis from Western Europe

Carla F. Murillo Perez, Alessio Gerussi, Palak J. Trivedi, Christophe Corpechot, Adriaan J. Van Der Meer, Pier Maria Battezzati, Keith D. Lindor, Frederik Nevens, Kris V. Kowdley, Tony Bruns, Nora Cazzagon, Annarosa Floreani, Atsushi Tanaka, Xiong Ma, Andrew L. Mason, Aliya Gulamhusein, Cyriel Y. Ponsioen, Marco Carbone, Ana Lleo, Marlyn J. MayoGeorge N. Dalekos, Nikolaos K. Gatselis, Douglas Thorburn, Xavier Verhelst, Albert Parés, Harry L.A. Janssen, Gideon M. Hirschfield, Bettina E. Hansen, Pietro Invernizzi, Willem J. Lammers*

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review


Background and aims The are geographic variations in the incidence and prevalence of primary biliary cholangitis (PBC). The aim was to explore whether clinical outcomes of patients within Western Europe differ according to geographical region. Methods Ursodeoxycholic acid-treated patients from European centers from the Global PBC database diagnosed from 1990 onwards were included. Patients with a time lag > 1 year from diagnosis to start of follow-up were excluded. Differences in baseline characteristics were studied according to North/South and East/West, whereas outcomes (transplant-free survival and decompensation) were studied with center latitude and longitude. Cox regression analyses were adjusted for age, sex, diagnosis year, biochemical markers, and cirrhosis as a time-dependent covariate. Results One thousand eight hundred seventy-eight patients were included, and there were no geographical differences in age or sex, with a mean age of 54 years and 89% female patients. Those in North Europe were more often of a moderately advanced/advanced Rotterdam biochemical stage (28.4%) compared with South Europe (20.6%). Additionally, they exhibited higher median alkaline phosphatase (2.0 ×ULN vs. 1.4 ×ULN) and transaminases. In multivariable analysis, there was a significant interaction between center latitude and longitude for decompensation (P < 0.001) and a trend for transplant-free survival, in which the Northwestern area demonstrated an increased risk for poor outcomes as compared to the reference (Paris). Conclusion We describe geographic variations in outcomes for patients across Europe from specialist centers in the Global PBC Study Group. Further study is important to explore the potential individual, environmental, and healthcare-related factors that may be contributors.

Original languageEnglish
Pages (from-to)112-119
Number of pages8
JournalEuropean Journal of Gastroenterology and Hepatology
Issue number1
Publication statusPublished - 1 Jan 2023

Bibliographical note

This study was performed on behalf of the Global PBC Study Group and was supported by unrestricted grants from Cymabay Therapeutics Inc., Intercept Pharmaceuticals, and previously from Zambon Nederland BV, and was funded by the Toronto General & Western Hospital Foundation (a not-for-profit organization) in Toronto, Canada, and the Foundation for Liver and Gastrointestinal Research (a not-for-profit organization) in Rotterdam, The Netherlands. The supporting parties had no influence on the study design, data collection and analyses, writing of the manuscript, or on the decision to submit the manuscript for publication.

A. Gerussi., M.C., and P.I. were partially supported by the grants from the Italian Ministry of Health (GR-2018-12367794 and PE-2016-02363915). This research was partially supported by the Italian Ministry of University and Research (MIUR) - Department of Excellence project PREMIA (PREcision MedIcine Approach: bringing biomarker research to clinic). A.G., M.C., and P.I. thank AMAF Monza ONLUS and AIRCS for the unrestricted research funding.

Publisher Copyright: © 2023 Lippincott Williams and Wilkins. All rights reserved.


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