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Factors Associated With Progression and Outcomes of Early Stage Primary Biliary Cholangitis.

Authors
  • Gatselis, Nikolaos K1
  • Goet, Jorn C2
  • Zachou, Kalliopi3
  • Lammers, Willem J2
  • Janssen, Harry L A4
  • Hirschfield, Gideon5
  • Corpechot, Christophe6
  • Lindor, Keith D7
  • Invernizzi, Pietro8
  • Mayo, Marlyn J9
  • Battezzati, Pier Maria10
  • Floreani, Annarosa11
  • Pares, Albert12
  • Lygoura, Vasiliki13
  • Nevens, Frederik14
  • Mason, Andrew L15
  • Kowdley, Kris V16
  • Ponsioen, Cyriel Y17
  • Bruns, Tony18
  • Thorburn, Douglas19
  • And 5 more
  • 1 Department of Medicine and Research Laboratory of Internal Medicine, School of Medicine, Thessaly University, Larissa, Greece. Electronic address: [email protected] , (Greece)
  • 2 Gastroenterology and Hepatology, Erasmus University Medical Center, Rotterdam, the Netherlands. , (Netherlands)
  • 3 Department of Medicine and Research Laboratory of Internal Medicine, School of Medicine, Thessaly University, Larissa, Greece. , (Greece)
  • 4 Toronto Centre for Liver Disease, Toronto General Hospital, University of Toronto, Toronto, Canada. , (Canada)
  • 5 National Institute for Health Research (NIHR) Biomedical Research Unit and Centre for Liver Research, University of Birmingham, Birmingham, United Kingdom. , (United Kingdom)
  • 6 Centre de Référence des Maladies Inflammatoires des Voies Biliaires, Hoôpital Saint-Antoine, Paris, France. , (France)
  • 7 Department of Gastroenterology and Hepatology, Mayo Clinic, Rochester, Minnesota; Arizona State University, Phoenix, Arizona.
  • 8 Division of Gastroenterology and Center for Autoimmune Liver Diseases, Department of Medicine and Surgery, University of Milan Bicocca, Milan, Italy. , (Italy)
  • 9 Digestive and Liver Diseases, University of Texas Southwestern Medical Center, Dallas, Texas.
  • 10 Department of Health Sciences, Università; degli Studi di Milano, Milan, Italy. , (Italy)
  • 11 Department of Surgery, Oncology and Gastroenterology, University of Padua, Padua, Italy. , (Italy)
  • 12 Liver Unit, Hospital Clinic, Centro de Investigación Biomédica en Red de Enfermedades Hepáticas y Digestivas (CIBERehd), Institut D'Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), University of Barcelona, Barcelona, Spain. , (Spain)
  • 13 Department of Medicine and Research Laboratory of Internal Medicine, School of Medicine, Thessaly University, Larissa, Greece; Division of Gastroenterology and Center for Autoimmune Liver Diseases, Department of Medicine and Surgery, University of Milan Bicocca, Milan, Italy. , (Greece)
  • 14 Department of Hepatology, University Hospitals Leuven, Katholieke Universiteit (KU) Leuven, Leuven, Belgium. , (Belgium)
  • 15 Division of Gastroenterology and Hepatology, University of Alberta, Edmonton, Alberta, Canada. , (Canada)
  • 16 Liver Care Network, Swedish Medical Center, Seattle, Washington.
  • 17 Department of Gastroenterology and Hepatology, Academic Medical Center, Amsterdam, the Netherlands. , (Netherlands)
  • 18 Department of Internal Medicine IV, Jena University Hospital, Friedrich Schiller University, Jena, Germany; Department of Medicine III, University Hospital Rheinisch Westfälische Technische Hochschule (RWTH) Aachen, Aachen, Germany. , (Germany)
  • 19 The Sheila Sherlock Liver Centre, The Royal Free Hospital, London, United Kingdom. , (United Kingdom)
  • 20 Department of Gastroenterology and Hepatology, Ghent University Hospital, Ghent, Belgium. , (Belgium)
  • 21 Gastroenterology and Hepatology, Erasmus University Medical Center, Rotterdam, the Netherlands; Toronto Centre for Liver Disease, Toronto General Hospital, University of Toronto, Toronto, Canada; Institute of Health Policy, Management and Evaluation, University of Toronto, Toronto, Canada. , (Canada)
Type
Published Article
Journal
Clinical gastroenterology and hepatology : the official clinical practice journal of the American Gastroenterological Association
Publication Date
Mar 01, 2020
Volume
18
Issue
3
Identifiers
DOI: 10.1016/j.cgh.2019.08.013
PMID: 31419573
Source
Medline
Keywords
Language
English
License
Unknown

Abstract

Patients usually receive a diagnosis of primary biliary cholangitis (PBC) at an early stage, based on biochemical analyses. We investigated the proportion of patients who progress to moderate or advanced PBC and factors associated with progression and patient survival. We obtained data from 1615 patients (mean age, 55.4 y) with early stage PBC (based on their normal levels of albumin and bilirubin), collected at the time of initial evaluation or treatment, from the Global PBC Study Group database (comprising patients at 19 liver centers in North American and European countries). We collected data from health care evaluations on progression to moderate PBC (abnormal level of bilirubin or albumin) or advanced-stage PBC (abnormal level of both). The median follow-up time was 7.9 years. The composite end point was decompensation, hepatocellular carcinoma, liver transplantation, or death. Of the 1615 patients identified with early stage PBC, 904 developed moderate PBC and 201 developed advanced disease over the study period. The proportions of patients who transitioned to moderate PBC at 1, 3, and 5 years were 12.9%, 30.2%, and 45.8%. The proportions of these patients who then transitioned to advanced PBC at 1, 3, and 5 years later were 3.4%, 12.5%, and 16.0%, respectively. During the follow-up period, 236 patients had a clinical event. The proportions of patients with moderate PBC and event-free survival were 97.9%, 95.1%, and 91.5% at 1, 3, and 5 years, respectively, and the proportions of patients with advanced PBC and event-free survival were 90.6%, 71.2%, and 58.3% at 1, 3, and 5 years later, respectively. Variables associated with transition from early to moderate PBC included baseline levels of bilirubin, albumin, and alkaline phosphatase; aspartate to alanine aminotransferase ratio; platelet count; and treatment with ursodeoxycholic acid. Transitions from early to moderate PBC and from moderate to advanced PBC were associated with higher probabilities of a clinical event (time-dependent hazard ratios, 3.0; 95% CI, 2.0-4.5; and 4.6; 95% CI, 3.5-6.2). Approximately half of patients with early stage PBC progress to a more severe stage within 5 years. Progression is associated with an increased risk of a clinical event, so surveillance is important for patients with early stage PBC. Copyright © 2020 AGA Institute. Published by Elsevier Inc. All rights reserved.

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