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

KA. Overbeek, JL. Poulsen, M. Lanzillotta, O. Vinge-Holmquist, P. Macinga, AF. Demirci, DP. Sindhunata, J. Backhus, H. Algül, J. Buijs, P. Levy, M. Kiriukova, E. Goni, M. Hollenbach, RC. Miksch, L. Kunovsky, M. Vujasinovic, S. Nikolic, L....

. 2024 ; 22 (5) : 994-1004.e10. [pub] 20240105

Jazyk angličtina Země Spojené státy americké

Typ dokumentu časopisecké články, práce podpořená grantem, multicentrická studie

Perzistentní odkaz   https://www.medvik.cz/link/bmc24014193

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.

1 M Sechenov 1st Moscow State Medical University Moscow Russia

2nd Department of Internal Medicine Gastroenterology and Geriatrics University Hospital Olomouc Faculty of Medicine and Dentistry Palacky University Olomouc Czech Republic

Acibadem Mehmet Ali Aydinlar University School of Medicine Istanbul Turkey

Biocruces Grupo Transplante Hepático Osakidetza Hospital Universitario Cruces Servicio Aparato Digestivo Barakaldo Spain

Central European Institute of Technology Masaryk University Brno Czech Republic

Centre for Pancreatic Diseases Department of Gastroenterology and Hepatology Aalborg University Hospital Aalborg Denmark

Department for Internal Medicine and Gastroenterology University Hospital Klinikum Oldenburg AöR Oldenburg Germany

Department of Digestive Surgery Akershus University Hospital Loerenskog Norway

Department of Digestive Surgery St Olavs Hospital Trondheim University Hospital Trondheim Norway

Department of Gastroenterology and Digestive Endoscopy Masaryk Memorial Cancer Center Institute Faculty of Medicine Masaryk University Brno Czech Republic

Department of Gastroenterology and Digestive Endoscopy Masaryk Memorial Cancer Institute Brno Czech Republic

Department of Gastroenterology and Endocrinology Philipps University Marburg Marburg Germany

Department of Gastroenterology and Hepatology Amsterdam UMC University of Amsterdam Amsterdam The Netherlands

Department of Gastroenterology and Hepatology Erasmus MC University Medical Center Rotterdam The Netherlands

Department of Gastroenterology and Hepatology Institute for Clinical and Experimental Medicine Prague Czech Republic

Department of Gastroenterology and Hepatology University Medical Center Utrecht Utrecht The Netherlands

Department of Gastroenterology Istanbul University Istanbul Faculty of Medicine Istanbul Turkey

Department of General Visceral and Transplantation Surgery University Hospital Ludwig Maximilian University Munich Munich Germany

Department of Infection Immunity and Cardiovascular Disease University of Sheffield Sheffield United Kingdom

Department of Internal Medicine 1 Martin Luther University Halle Germany

Department of Internal Medicine 1 University Hospital Schleswig Holstein Kiel Germany

Department of Internal Medicine 1 University Hospital Ulm Ulm Germany

Department of Internal Medicine Marmara University Research and Education Hospital Istanbul Turkey

Department of Medicine 1 Deutsches Zentrum Immuntherapie Kussmaul Campus for Medical Research University Erlangen Nürnberg Erlangen Germany

Department of Medicine 2 Technische Universität München München Germany

Department of Medicine 2 University Hospital Ludwig Maximilian University Munich Munich Germany

Department of Medicine 2 University Medical Center Mannheim Medical Faculty at Mannheim University of Heidelberg Mannheim Germany

Department of Medicine A University Medicine Greifswald Greifswald Germany

Department of Molecular and Clinical Cancer Medicine Institute of Translational Medicine University of Liverpool Liverpool United Kingdom

Department of Outpatient Therapy and Family Medicine Tver State Medical University Tver Russia

Department of Pediatrics University Hospital Brno Faculty of Medicine Masaryk University Brno Czech Republic

Department of Surgery University Hospital Brno Faculty of Medicine Masaryk University Brno Czech Republic

Department of Upper Abdominal Diseases Karolinska University Hospital Stockholm Sweden

Department of Upper Gastrointestinal Pancreatic and Biliary Diseases A S Loginov Moscow Clinical Research Center Moscow Russia

Division of Gastroenterology and Hepatology Department of Internal Medicine Essen University Hospital University of Duisberg Essen Essen Germany

Division of Gastroenterology Medical Department 2 Oncology Gastroenterology Hepatology Pulmonology Infectious Diseases University of Leipzig Medical Center Leipzig Germany

Division of Pancreatic Diseases Heart and Vascular Centre Semmelweis University Budapest Hungary

Division of Pancreatic Surgery Pancreas Translational and Clinical Research Center San Raffaele Scientific Institute IRCCS Vita Salute San Raffaele University Milan Italy

Endoscopy Service Department of Diagnostic and Therapeutic Services Mediterranean Institute for Transplantation and Advanced Specialized Therapies Palermo Italy

Faculty of Medicine Campus Ahus University of Oslo Oslo Norway

Gastrointestinal Endoscopy Unit Humanitas Mater Domini Castellanza Italy

HPB Unit Freeman Hospital Newcastle Upon Tyne United Kingdom

Institute for Translational Medicine Szentágothai Research Centre Medical School University of Pécs Pécs Hungary

Pancreato Biliary Endoscopy and Endosonography Division Pancreas Translational and Clinical Research Center San Raffaele Scientific Institute IRCCS Vita Salute San Raffaele University Milan Italy

Pancreatology Unit APHP Beaujon Hospital Clichy France

Translational Gastroenterology Unit John Radcliffe Hospital and Nuffield Department of Medicine University of Oxford Oxford United Kingdom

Unit of Immunology Rheumatology Allergy and Rare Diseases San Raffaele Scientific Institute Milan Italy

Citace poskytuje Crossref.org

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$a Overbeek, Kasper A $u Department of Gastroenterology & Hepatology, Erasmus MC, University Medical Center, Rotterdam, The Netherlands. Electronic address: k.overbeek@erasmusmc.nl
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$a Type 1 Autoimmune Pancreatitis in Europe: Clinical Profile and Response to Treatment / $c KA. Overbeek, JL. Poulsen, M. Lanzillotta, O. Vinge-Holmquist, P. Macinga, AF. Demirci, DP. Sindhunata, J. Backhus, H. Algül, J. Buijs, P. Levy, M. Kiriukova, E. Goni, M. Hollenbach, RC. Miksch, L. Kunovsky, M. Vujasinovic, S. Nikolic, L. Dickerson, M. Hirth, MF. Neurath, M. Zumblick, J. Vila, M. Jalal, G. Beyer, F. Frost, S. Carrara, Z. Kala, P. Jabandziev, G. Sisman, F. Akyuz, G. Capurso, M. Falconi, A. Arlt, FP. Vleggaar, L. Barresi, B. Greenhalf, L. Czakó, P. Hegyi, A. Hopper, MK. Nayar, TM. Gress, F. Vitali, A. Schneider, CM. Halloran, J. Trna, AV. Okhlobystin, L. Dagna, DL. Cahen, D. Bordin, V. Rebours, J. Mayerle, A. Kahraman, S. Rasch, E. Culver, A. Kleger, E. Martínez-Moneo, O. Røkke, T. Hucl, SS. Olesen, MJ. Bruno, E. Della-Torre, U. Beuers, JM. Löhr, J. Rosendahl, PrescrAIP Study Group
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$a 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.
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$a Poulsen, Jakob L $u Centre for Pancreatic Diseases, Department of Gastroenterology & Hepatology, Aalborg University Hospital, Aalborg, Denmark
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$a Kiriukova, Mariia $u Department of Upper Gastrointestinal, Pancreatic, and Biliary Diseases, A.S. Loginov Moscow Clinical Research Center, Moscow, Russia
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$a Goni, Elisabetta $u Department of Medicine II, University Hospital, Ludwig Maximilian University Munich, Munich, Germany
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$a Hollenbach, Marcus $u Division of Gastroenterology, Medical Department II - Oncology, Gastroenterology, Hepatology, Pulmonology, Infectious Diseases, University of Leipzig Medical Center, Leipzig, Germany
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$a Miksch, Rainer C $u Department of General, Visceral, and Transplantation Surgery, University Hospital, Ludwig Maximilian University Munich, Munich, Germany
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$a Kunovsky, Lumir $u 2nd Department of Internal Medicine, Gastroenterology and Geriatrics, University Hospital Olomouc, Faculty of Medicine and Dentistry, Palacky University, Olomouc, Czech Republic; Department of Surgery, University Hospital Brno, Faculty of Medicine, Masaryk University, Brno, Czech Republic; Department of Gastroenterology and Digestive Endoscopy, Masaryk Memorial Cancer Institute, Brno, Czech Republic
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$a Vujasinovic, Miroslav $u Department of Upper Abdominal Diseases, Karolinska University Hospital, Stockholm, Sweden
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$a Sisman, Gurhan $u Acibadem Mehmet Ali Aydinlar University School of Medicine, Istanbul, Turkey
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$a Capurso, Gabriele $u Pancreato-Biliary Endoscopy & Endosonography Division, Pancreas Translational & Clinical Research Center, San Raffaele Scientific Institute IRCCS, Vita-Salute San Raffaele University, Milan, Italy
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$a Falconi, Massimo $u Division of Pancreatic Surgery, Pancreas Translational & Clinical Research Center, San Raffaele Scientific Institute IRCCS, Vita-Salute San Raffaele University, Milan, Italy
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$a Vleggaar, Frank P $u Department of Gastroenterology and Hepatology, University Medical Center Utrecht, Utrecht, The Netherlands
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$a Hopper, Andrew $u Department of Infection, Immunity and Cardiovascular Disease, University of Sheffield, Sheffield, United Kingdom
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$a Gress, Thomas M $u Department of Gastroenterology and Endocrinology, Philipps-University Marburg, Marburg, Germany
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700    1_
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$a Rebours, Vinciane $u Pancreatology Unit, APHP Beaujon Hospital, Clichy, France
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700    1_
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700    1_
$a Rosendahl, Jonas $u Department of Internal Medicine I, Martin Luther University, Halle (Saale), Germany. Electronic address: Jonas.rosendahl@uk-halle.de
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$a PrescrAIP Study Group
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