Carla F Murillo Perez1,2, Jorn C Goet3, Willem J Lammers3, Aliya Gulamhusein1, Henk R van Buuren3, Cyriel Y Ponsioen4, Marco Carbone5, Andrew Mason6, Christophe Corpechot7, Pietro Invernizzi5, Marlyn J Mayo8, Pier Maria Battezzati9, Annarosa Floreani10, Albert Pares11, Frederik Nevens12, Kris V Kowdley13, Tony Bruns14, George N Dalekos15, Douglas Thorburn16, Gideon Hirschfield17, Nicholas F LaRusso18, Keith D Lindor19, Kalliopi Zachou15, Raoul Poupon7, Palak J Trivedi17, Xavier Verhelst20, Harry L A Janssen1, Bettina E Hansen1,3,21. 1. Toronto Centre for Liver Disease, Toronto General Hospital, University Health Network, Toronto, ON, Canada. 2. Insitute of Medical Science, University of Toronto, Toronto, ON, Canada. 3. Gastroenterology and Hepatology, Erasmus University Medical Center, Rotterdam, The Netherlands. 4. Department of Gastroenterology and Hepatology, Academic Medical Center, Amsterdam, The Netherlands. 5. Division of Gastroenterology and Center for Autoimmune Liver Diseases, Department of Medicine and Surgery, University of Milan-Bicocca, Milan, Italy. 6. Divison of Gastroenterology and Hepatology, University of Alberta, Edmonton, AB, Canada. 7. Centre de Référence des Maladies Inflammatoires des VoiesBiliaires, Hôpital Saint-Antoine, Paris, France. 8. Digestive and Liver diseases, UT Southwestern Medical Center, Dallas, TX. 9. Department of Health Sciences, Università degli Studi di Milano, Milan, Italy. 10. Department of Surgery, Oncology and Gastroenterology, University of Padua, Padua, Italy. 11. Liver Unit, Hospital Clínic, CIBERehd, IDIBAPS, University of Barcelona, Barcelona, Spain. 12. Department of Hepatology, University Hospitals Leuven, KU Leuven, Leuven, Belgium. 13. Liver Care Network, Swedish Medical Center, Seattle, WA. 14. Department of Internal Medicine IV, Jena University Hospital, Friedrich Schiller University of Jena, Jena, Germany. 15. Department of Medicine and Research Laboratory of Internal Medicine, University of Thessaly, Larissa, Greece. 16. The Sheila Sherlock Liver Centre, The Royal Free Hospital, London, UK. 17. NIHR, Biomedical Research Centre and Centre for Liver Research, University of Birmingham, Birmingham, UK. 18. Division of Gastroenterology and Hepatology, Mayo Clinic, Rochester, MN. 19. Arizona State University, Phoenix, AZ. 20. Department of Gastroenterology and Hepatology, Ghent University Hospital, Ghent, Belgium. 21. Institute of Health Policy, Management and Evaluation, University of Toronto, Toronto, ON, Canada.
Abstract
Changes over time in the presenting features and clinical course of patients with primary biliary cholangitis are poorly described. We sought to describe temporal trends in patient and disease characteristics over a 44-year period across a large international primary biliary cholangitis cohort of 4,805 patients diagnosed between 1970 and 2014, from 17 centers across Europe and North America. Patients were divided into five cohorts according to their year of diagnosis: 1970-1979 (n = 143), 1980-1989 (n = 858), 1990-1999 (n = 1,754), 2000-2009 (n = 1,815), and ≥2010 (n = 235). Age at diagnosis, disease stage, response to ursodeoxycholic acid, and clinical outcomes were compared. Mean age at diagnosis increased incrementally by 2-3 years per decade from 46.9 ± 10.1 years in the 1970s to 57.0 ± 12.1 years from 2010 onward (P < 0.001). The female to male ratio (9:1) and antimitochondrial antibody positivity (90%) were not significantly variable. The proportion of patients presenting with mild biochemical disease (according to Rotterdam staging) increased from 41.3% in the 1970s to 72.2% in the 1990s (P < 0.001) and remained relatively stable thereafter. Patients with a mild histological stage at diagnosis increased from 60.4% (1970-1989) to 76.5% (1990-2014) (P < 0.001). Correspondingly, response to ursodeoxycholic acid according to Paris-I criteria increased; 51.7% in the 1970s and 70.5% in the 1990s (P < 0.001). Recent decades were also characterized by lower decompensation rates (18.5% in the 1970s to 5.8% in the 2000s, P < 0.001) and higher 10-year transplant-free survival (48.4%, 68.7%, 79.7%, and 80.1% for each respective cohort; P < 0.001). CONCLUSION: In recent decades, a pattern of primary biliary cholangitis presentation consistent with an older age at diagnosis alongside reduced disease severity has been noted; the observed trends may be explained by an increase in routine testing of liver function and/or a changing environmental trigger. (Hepatology 2018;67:1920-1930).
Changes over time in the presenting features and clinical course of patients with primary biliary cholangitis are poorly described. We sought to describe temporal trends in patient and disease characteristics over a 44-year period across a large international primary biliary cholangitis cohort of 4,805 patients diagnosed between 1970 and 2014, from 17 centers across Europe and North America. Patients were divided into five cohorts according to their year of diagnosis: 1970-1979 (n = 143), 1980-1989 (n = 858), 1990-1999 (n = 1,754), 2000-2009 (n = 1,815), and ≥2010 (n = 235). Age at diagnosis, disease stage, response to ursodeoxycholic acid, and clinical outcomes were compared. Mean age at diagnosis increased incrementally by 2-3 years per decade from 46.9 ± 10.1 years in the 1970s to 57.0 ± 12.1 years from 2010 onward (P < 0.001). The female to male ratio (9:1) and antimitochondrial antibody positivity (90%) were not significantly variable. The proportion of patients presenting with mild biochemical disease (according to Rotterdam staging) increased from 41.3% in the 1970s to 72.2% in the 1990s (P < 0.001) and remained relatively stable thereafter. Patients with a mild histological stage at diagnosis increased from 60.4% (1970-1989) to 76.5% (1990-2014) (P < 0.001). Correspondingly, response to ursodeoxycholic acid according to Paris-I criteria increased; 51.7% in the 1970s and 70.5% in the 1990s (P < 0.001). Recent decades were also characterized by lower decompensation rates (18.5% in the 1970s to 5.8% in the 2000s, P < 0.001) and higher 10-year transplant-free survival (48.4%, 68.7%, 79.7%, and 80.1% for each respective cohort; P < 0.001). CONCLUSION: In recent decades, a pattern of primary biliary cholangitis presentation consistent with an older age at diagnosis alongside reduced disease severity has been noted; the observed trends may be explained by an increase in routine testing of liver function and/or a changing environmental trigger. (Hepatology 2018;67:1920-1930).
Authors: Maren H Harms; Quisette P Janssen; Rene Adam; Christophe Duvoux; Darius Mirza; Ernest Hidalgo; Christopher Watson; Stephen J Wigmore; Massimo Pinzani; Helena Isoniemi; Johann Pratschke; Krzysztof Zieniewicz; Jurgen L Klempnauer; William Bennet; Vincent Karam; Henk R van Buuren; Bettina E Hansen; Herold J Metselaar Journal: Aliment Pharmacol Ther Date: 2018-12-18 Impact factor: 8.171
Authors: Melissa Palmer; Arie Regev; Keith Lindor; Mark I Avigan; Lara Dimick-Santos; William Treem; John F Marcinak; James H Lewis; Frank A Anania; Daniel Seekins; Benjamin L Shneider; Naga Chalasani Journal: Aliment Pharmacol Ther Date: 2019-11-25 Impact factor: 8.171