Type 1 Autoimmune Pancreatitis in Europe: Clinical Profile and Response to Treatment.

Autor: Overbeek KA; Department of Gastroenterology & Hepatology, Erasmus MC, University Medical Center, Rotterdam, The Netherlands. Electronic address: k.overbeek@erasmusmc.nl., Poulsen JL; Centre for Pancreatic Diseases, Department of Gastroenterology & Hepatology, Aalborg University Hospital, Aalborg, Denmark., Lanzillotta M; Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), San Raffaele Scientific Institute, Milan, Italy., Vinge-Holmquist O; Department of Digestive Surgery, Akershus University Hospital, Loerenskog, Norway; Department of Digestive Surgery, St. Olavs Hospital, Trondheim University Hospital, Trondheim, Norway., Macinga P; Department of Gastroenterology and Hepatology, Institute for Clinical and Experimental Medicine, Prague, Czech Republic., Demirci AF; Department of Internal Medicine, Marmara University Research and Education Hospital, Istanbul, Turkey., Sindhunata DP; Department of Gastroenterology & Hepatology, Amsterdam UMC, University of Amsterdam, Amsterdam, The Netherlands., Backhus J; Department of Internal Medicine I, University Hospital Ulm, Ulm, Germany., Algül H; Department of Medicine II, Technische Universität München, München, Germany., Buijs J; Department of Gastroenterology & Hepatology, Erasmus MC, University Medical Center, Rotterdam, The Netherlands., Levy P; Pancreatology Unit, APHP Beaujon Hospital, Clichy, France., Kiriukova M; Department of Upper Gastrointestinal, Pancreatic, and Biliary Diseases, A.S. Loginov Moscow Clinical Research Center, Moscow, Russia., Goni E; Department of Medicine II, University Hospital, Ludwig Maximilian University Munich, Munich, Germany., Hollenbach M; Division of Gastroenterology, Medical Department II - Oncology, Gastroenterology, Hepatology, Pulmonology, Infectious Diseases, University of Leipzig Medical Center, Leipzig, Germany., Miksch RC; Department of General, Visceral, and Transplantation Surgery, University Hospital, Ludwig Maximilian University Munich, Munich, Germany., Kunovsky L; 2nd Department of Internal Medicine, Gastroenterology and Geriatrics, University Hospital Olomouc, Faculty of Medicine and Dentistry, Palacky University, Olomouc, Czech Republic; Department of Surgery, University Hospital Brno, Faculty of Medicine, Masaryk University, Brno, Czech Republic; Department of Gastroenterology and Digestive Endoscopy, Masaryk Memorial Cancer Institute, Brno, Czech Republic., Vujasinovic M; Department of Upper Abdominal Diseases, Karolinska University Hospital, Stockholm, Sweden., Nikolic S; Department of Upper Abdominal Diseases, Karolinska University Hospital, Stockholm, Sweden., Dickerson L; Department of Molecular and Clinical Cancer Medicine, Institute of Translational Medicine, University of Liverpool, Liverpool, United Kingdom., Hirth M; Department of Medicine II, University Medical Center Mannheim, Medical Faculty at Mannheim, University of Heidelberg, Mannheim, Germany., Neurath MF; Department of Medicine I, Deutsches Zentrum Immuntherapie (DZI), Kussmaul Campus for Medical Research, University Erlangen-Nürnberg, Erlangen, Germany., Zumblick M; Department of Gastroenterology and Endocrinology, Philipps-University Marburg, Marburg, Germany., Vila J; HPB Unit, Freeman Hospital, Newcastle Upon Tyne, United Kingdom., Jalal M; Department of Infection, Immunity and Cardiovascular Disease, University of Sheffield, Sheffield, United Kingdom., Beyer G; Department of Medicine II, University Hospital, Ludwig Maximilian University Munich, Munich, Germany., Frost F; Department of Medicine A, University Medicine Greifswald, Greifswald, Germany., Carrara S; Gastrointestinal Endoscopy Unit, Humanitas Mater Domini, Castellanza, Italy., Kala Z; Department of Surgery, University Hospital Brno, Faculty of Medicine, Masaryk University, Brno, Czech Republic., Jabandziev P; Department of Pediatrics, University Hospital Brno, Faculty of Medicine, Masaryk University, Brno, Czech Republic; Central European Institute of Technology, Masaryk University, Brno, Czech Republic., Sisman G; Acibadem Mehmet Ali Aydinlar University School of Medicine, Istanbul, Turkey., Akyuz F; Department of Gastroenterology, Istanbul University, Istanbul Faculty of Medicine, Istanbul, Turkey., Capurso G; Pancreato-Biliary Endoscopy & Endosonography Division, Pancreas Translational & Clinical Research Center, San Raffaele Scientific Institute IRCCS, Vita-Salute San Raffaele University, Milan, Italy., Falconi M; Division of Pancreatic Surgery, Pancreas Translational & Clinical Research Center, San Raffaele Scientific Institute IRCCS, Vita-Salute San Raffaele University, Milan, Italy., Arlt A; Department of Internal Medicine I, University Hospital Schleswig-Holstein, Kiel, Germany; Department for Internal Medicine and Gastroenterology, University Hospital, Klinikum Oldenburg AöR, Oldenburg, Germany., Vleggaar FP; Department of Gastroenterology and Hepatology, University Medical Center Utrecht, Utrecht, The Netherlands., Barresi L; Endoscopy Service, Department of Diagnostic and Therapeutic Services, Mediterranean Institute for Transplantation and Advanced Specialized Therapies (IRCSS-ISMETT), Palermo, Italy., Greenhalf B; Department of Molecular and Clinical Cancer Medicine, Institute of Translational Medicine, University of Liverpool, Liverpool, United Kingdom., Czakó L; Institute for Translational Medicine, Szentágothai Research Centre, Medical School, University of Pécs, Pécs, Hungary., Hegyi P; Institute for Translational Medicine, Szentágothai Research Centre, Medical School, University of Pécs, Pécs, Hungary; Division of Pancreatic Diseases, Heart and Vascular Centre, Semmelweis University, Budapest, Hungary., Hopper A; Department of Infection, Immunity and Cardiovascular Disease, University of Sheffield, Sheffield, United Kingdom., Nayar MK; HPB Unit, Freeman Hospital, Newcastle Upon Tyne, United Kingdom., Gress TM; Department of Gastroenterology and Endocrinology, Philipps-University Marburg, Marburg, Germany., Vitali F; Department of Medicine I, Deutsches Zentrum Immuntherapie (DZI), Kussmaul Campus for Medical Research, University Erlangen-Nürnberg, Erlangen, Germany., Schneider A; Department of Medicine II, University Medical Center Mannheim, Medical Faculty at Mannheim, University of Heidelberg, Mannheim, Germany., Halloran CM; Department of Molecular and Clinical Cancer Medicine, Institute of Translational Medicine, University of Liverpool, Liverpool, United Kingdom., Trna J; Department of Gastroenterology and Digestive Endoscopy, Masaryk Memorial Cancer Center Institute, Faculty of Medicine, Masaryk University, Brno, Czech Republic., Okhlobystin AV; I.M. Sechenov First Moscow State Medical University, Moscow, Russia., Dagna L; Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), San Raffaele Scientific Institute, Milan, Italy., Cahen DL; Department of Gastroenterology & Hepatology, Erasmus MC, University Medical Center, Rotterdam, The Netherlands., Bordin D; Department of Upper Gastrointestinal, Pancreatic, and Biliary Diseases, A.S. Loginov Moscow Clinical Research Center, Moscow, Russia; Department of Outpatient Therapy and Family Medicine, Tver State Medical University, Tver, Russia., Rebours V; Pancreatology Unit, APHP Beaujon Hospital, Clichy, France., Mayerle J; Department of Medicine II, University Hospital, Ludwig Maximilian University Munich, Munich, Germany., Kahraman A; Division of Gastroenterology and Hepatology, Department of Internal Medicine, Essen University Hospital, University of Duisberg-Essen, Essen, Germany., Rasch S; Department of Medicine II, Technische Universität München, München, Germany., Culver E; Translational Gastroenterology Unit, John Radcliffe Hospital and Nuffield Department of Medicine, University of Oxford, Oxford, United Kingdom., Kleger A; Department of Internal Medicine I, University Hospital Ulm, Ulm, Germany., Martínez-Moneo E; Biocruces, Grupo Transplante Hepático, Osakidetza, Hospital Universitario Cruces, Servicio Aparato Digestivo, Barakaldo, Spain., Røkke O; Department of Digestive Surgery, Akershus University Hospital, Loerenskog, Norway; Faculty of Medicine, Campus Ahus, University of Oslo, Oslo, Norway., Hucl T; Department of Gastroenterology and Hepatology, Institute for Clinical and Experimental Medicine, Prague, Czech Republic., Olesen SS; Centre for Pancreatic Diseases, Department of Gastroenterology & Hepatology, Aalborg University Hospital, Aalborg, Denmark., Bruno MJ; Department of Gastroenterology & Hepatology, Erasmus MC, University Medical Center, Rotterdam, The Netherlands., Della-Torre E; Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), San Raffaele Scientific Institute, Milan, Italy., Beuers U; Department of Gastroenterology & Hepatology, Amsterdam UMC, University of Amsterdam, Amsterdam, The Netherlands., Löhr JM; 2nd Department of Internal Medicine, Gastroenterology and Geriatrics, University Hospital Olomouc, Faculty of Medicine and Dentistry, Palacky University, Olomouc, Czech Republic; Department of Surgery, University Hospital Brno, Faculty of Medicine, Masaryk University, Brno, Czech Republic; Department of Gastroenterology and Digestive Endoscopy, Masaryk Memorial Cancer Institute, Brno, Czech Republic., Rosendahl J; Department of Internal Medicine I, Martin Luther University, Halle (Saale), Germany. Electronic address: Jonas.rosendahl@uk-halle.de.
Jazyk: angličtina
Zdroj: Clinical gastroenterology and hepatology : the official clinical practice journal of the American Gastroenterological Association [Clin Gastroenterol Hepatol] 2024 May; Vol. 22 (5), pp. 994-1004.e10. Date of Electronic Publication: 2024 Jan 05.
DOI: 10.1016/j.cgh.2023.12.010
Abstrakt: Background & Aims: Autoimmune pancreatitis (AIP) is an immune-mediated disease of the pancreas with distinct pathophysiology and manifestations. Our aims were to characterize type 1 AIP in a large pan-European cohort and study the effectiveness of current treatment regimens.
Methods: We retrospectively analyzed adults diagnosed since 2005 with type 1 or not-otherwise-specified AIP in 42 European university hospitals. Type 1 AIP was uniformly diagnosed using specific diagnostic criteria. Patients with type 2 AIP and those who had undergone pancreatic surgery were excluded. The primary end point was complete remission, defined as the absence of clinical symptoms and resolution of the index radiologic pancreatic abnormalities attributed to AIP.
Results: We included 735 individuals with AIP (69% male; median age, 57 years; 85% White). Steroid treatment was started in 634 patients, of whom 9 (1%) were lost to follow-up. The remaining 625 had a 79% (496/625) complete, 18% (111/625) partial, and 97% (607/625) cumulative remission rate, whereas 3% (18/625) did not achieve remission. No treatment was given in 95 patients, who had a 61% complete (58/95), 19% partial (18/95), and 80% cumulative (76/95) spontaneous remission rate. Higher (≥0.4 mg/kg/day) corticosteroid doses were no more effective than lower (<0.4 mg/kg/day) doses (odds ratio, 0.428; 95% confidence interval, 0.054-3.387) and neither was a starting dose duration >2 weeks (odds ratio, 0.908; 95% confidence interval, 0.818-1.009). Elevated IgG4 levels were independently associated with a decreased chance of complete remission (odds ratio, 0.639; 95% confidence interval, 0.427-0.955). Relapse occurred in 30% of patients. Relapses within 6 months of remission induction were independent of the steroid-tapering duration, induction treatment duration, and total cumulative dose.
Conclusions: Patients with type 1 AIP and elevated IgG4 level may need closer monitoring. For remission induction, a starting dose of 0.4 mg/kg/day for 2 weeks followed by a short taper period seems effective. This study provides no evidence to support more aggressive regimens.
(Copyright © 2024 The Author(s). Published by Elsevier Inc. All rights reserved.)
Databáze: MEDLINE