Epidemiology, clinical features and management of autoimmune hepatitis in Switzerland : a retrospective and prospective cohort study

Article Article scientifique Article Epidemiology, clinical features and management of autoimmune hepatitis in Switzerland : a retrospective and prospective cohort study eng 2023-08-31 2023-08-31 author Ludz Christine Facoltà di Scienze Biomediche, Università della Svizzera Italiana, Lugano, Switzerland. author Stirnimann Guido Universitätsklinik für Viszerale Chirurgie und Medizin, Inselspital Bern, Bern, Switzerland. author Semela David Klinik für Gastroenterologie und Hepatologie, Kantonsspital St. Gallen, St. Gallen, Switzerland. author Mertens Joachim GastroZentrum Hirslanden, Zürich, Switzerland. author Kremer Andreas E Klinik für Gastroenterologie und Hepatologie, Universitätsspital Zürich, Zürich, Switzerland. author Filipowicz Sinnreich Magdalena Clinic for Gastroenterology and Hepatology, Medizinische Universitätsklinik, Kantonsspital Baselland, Liestal, Switzerland. author Sokollik Christiane Departement Pädiatrische Gastroenterologie, Hepatologie und Ernährung, Kinderklinik Inselspital Bern, Bern Switzerland. author Bernsmeier Christine Gastroenterologie/Hepatologie, Universitäres Bauchzentrum Basel Clarunis, Basel, Switzerland. author Bresson Hadni Solange Service de Gastroentérologie and Hépatologie, Hôpitaux Universitaires de Genève, Geneva, Switzerland. 134717 author Mclin Valérie Anne Département de l'Enfant et de l'Adolescent, Centre Suisse des Maladies du Foie de l'Enfant, Hôpitaux Universitaires de Genève, Geneva, Switzerland. 799651 Département de Pédiatrie, Gynécologie et Obstétrique (PETRI) author Rock Nathalie Département de l'Enfant et de l'Adolescent, Centre Suisse des Maladies du Foie de l'Enfant, Hôpitaux Universitaires de Genève, Geneva, Switzerland. 826963 author Braegger Christian Division of Pediatric Gastroenterology and Nutrition, University Children's Hospital Zurich, Zurich, Switzerland. author Posovszky Carsten Nutrition Research Unit, University Children's Hospital Zurich, Zurich, Switzerland. author Müller Pascal Departement pädiatrische Gastroenterologie und Hepatologie, Ostschweizer Kinderspital, St. Gallen, Switzerland. author Cremer Matthias Departement für Kinder- und Jugendmedizin, Kantonsspital Graubünden, Chur, Switzerland. author De Gottardi Andrea Servizio di Gastroenterologia, Ente Ospedaliero Cantonale Ospedale Regionale di Lugano, Lugano, Switzerland. author Galante Antonio Servizio di Gastroenterologia, Ente Ospedaliero Cantonale Ospedale Regionale di Lugano, Lugano, Switzerland. author Furlano Raoul University Children's Hospital Basel, Paediatric Gastroenterology and Nutrition, Basel, Switzerland. author Righini-Grunder Franziska Departement pädiatrische Gastroenterologie, Luzerner Kantonsspital/Kinderspital, Lucerne, Switzerland. author Becker Björn Departement Innere Medizin, Klinik für Gastroenterologie und Hepatologie, Kantonsspital Winterthur, Winterthur, Switzerland. author Böhm Stephan Departement Gastroenterologie und Hepatologie, Klinik Innere Medizin, Spital Bülach, Bülach, Switzerland. author Heyland Klaas Departement Kindergastroenterologie, Kantonsspital Winterthur, Winterthur, Switzerland. author Nydegger Andreas Département femme-mère-enfant, Centre hospitalier universitaire Vaudois, Lausanne, Switzerland. author Limoni Costanzo University of Applied Sciences and Arts of Southern Switzerland, Lugano, Switzerland. author Vergani Diego MowatLabs, Faculty of Life Sciences and Medicine, King's College London, King's College Hospital, London, UK. author Mieli-Vergani Giorgina MowatLabs, Faculty of Life Sciences and Medicine, King's College London, King's College Hospital, London, UK. author Di Bartolomeo Claudia Epatocentro Ticino, Lugano, Switzerland. author Cerny Andreas Epatocentro Ticino, Lugano, Switzerland. author Terziroli Beretta-Piccoli Benedetta Facoltà di Scienze Biomediche, Università della Svizzera Italiana, Lugano, Switzerland.,MowatLabs, Faculty of Life Sciences and Medicine, King's College London, King's College Hospital, London, UK.,Epatocentro Ticino, Lugano, Switzerland. 40102 10.57187/smw.2023.40102 37769636 1424-3997 https://smw.ch/index.php/smw/article/view/3474 Background and aims: The Swiss Autoimmune Hepatitis Cohort Study is a nationwide registry, initiated in 2017, that collects retrospective and prospective clinical data and biological samples from patients of all ages with autoimmune hepatitis treated at Swiss hepatology centres. Here, we report the analysis of the first 5 years of registry data. Results: A total of 291 patients with autoimmune hepatitis have been enrolled, 30 of whom were diagnosed before 18 years of age and composed the paediatric cohort. Paediatric cohort: median age at diagnosis 12.5 years (range 1-17, interquartile range (IQR) 8-15), 16 (53%) girls, 6 (32%) with type 2 autoimmune hepatitis, 8 (27%) with autoimmune sclerosing cholangitis, 1 with primary biliary cholangitis variant syndrome, 4 (15%) with inflammatory bowel disease and 10 (41%) with advanced liver fibrosis at diagnosis. Adult cohort: median age at diagnosis 54 years (range 42-64, IQR 18-81), 185 (71%) women, 51 (20%) with primary biliary cholangitis variant syndrome, 22 (8%) with primary sclerosing cholangitis variant syndrome, 9 (4%) with inflammatory bowel disease and 66 (32%) with advanced liver fibrosis at diagnosis. The median follow-up time for the entire cohort was 5.2 years (IQR 3-9.3 years). Treatment in children: 29 (97%) children were initially treated with corticosteroids, 28 of whom received combination treatment with azathioprine. Budesonide was used in four children, all in combination with azathioprine. Mycophenolate mofetil was used in five children, all of whom had previously received corticosteroids and thiopurine. Treatment in adults (data available for 228 patients): 219 (96%) were treated with corticosteroids, mostly in combination with azathioprine. Predniso(lo)ne was the corticosteroid used in three-quarters of patients; the other patients received budesonide. A total of 78 (33%) patients received mycophenolate mofetil, 62 of whom had previously been treated with azathioprine. Complete biochemical response was achieved in 13 of 19 (68%) children and 137 of 182 (75%) adults with available follow-up data. All children were alive at the last follow-up, and none had undergone liver transplantation. Five (2%) adults underwent liver transplantation, two of whom had a fulminant presentation. Four (2%) adults with autoimmune hepatitis died (two from liver-associated causes). Conclusion: Patients with autoimmune hepatitis in Switzerland had clinical features similar to those in other cohorts. The proportion of patients diagnosed with primary biliary cholangitis variant syndrome was higher than expected. Autoimmune hepatitis was managed according to guidelines, except for the use of budesonide in a small proportion of paediatric patients. The outcomes were excellent, but the findings must be confirmed over a longer follow-up period. Adult Humans Child Female Infant Child, Preschool Adolescent Middle Aged Male Azathioprine / therapeutic use Retrospective Studies Hepatitis, Autoimmune / complications Hepatitis, Autoimmune / diagnosis Hepatitis, Autoimmune / drug therapy Prospective Studies Switzerland / epidemiology Cohort Studies Liver Cirrhosis, Biliary / complications Liver Cirrhosis, Biliary / drug therapy Mycophenolic Acid / therapeutic use Liver Cirrhosis Inflammatory Bowel Diseases / drug therapy Budesonide / therapeutic use Dewey 616 Dewey 618 58 Département de pédiatrie, gynécologie et obstétrique 223 56 Département de médecine 221 false 559 Pathologies hépatiques congénitales et acquises de l'enfant 900 Swiss medical weekly 153 318f4515-ad9b-42cc-bc8f-4c841722d9f7 Article (Published version) Public Article_scientifique_from_unpaywall.pdf 563428 application/pdf CC BY-4.0 a20586888e846c7f221d6ff277ce44a2e68fd251f36319023af9572e973de3a3 openAccess Faculté de médecine / Section de médecine clinique / Département de pédiatrie, gynécologie et obstétrique Faculté de médecine / Section de médecine clinique / Département de médecine

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