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

Overbeek KA, Poulsen JL, Lanzillotta M, Vinge-Holmquist O, Macinga P, Demirci AF, Sindhunata DP, Backhus J, Algül H, Buijs J, Levy P, Kiriukova M, Goni E, Hollenbach M, Miksch RC, Kunovsky L, Vujasinovic M, Nikolic S, Dickerson L, Hirth M, Neurath M, Zumblick M, Vila J, Jalal M, Beyer G, Frost F, Carrara S, Kala Z, Jabandziev P, Sisman G, Akyuz F, Capurso G, Falconi M, Arlt A, Vleggaar FP, Barresi L, Greenhalf B, Czakó L, Hegyi P, Hopper A, Nayar MK, Gress TM, Vitali F, Schneider A, Halloran CM, Trna J, Okhlobystin AV, Dagna L, Cahen DL, Bordin D, Rebours V, Mayerle J, Kahraman A, Rasch S, Culver E, Kleger A, Martínez-Moneo E, Røkke O, Hucl T, Olesen SS, Bruno MJ, Della-Torre E, Beuers U, Löhr JM, Rosendahl J, Drewes AM, Haas SL, Hoyer BF, Hampe J, Hinrichs CN, Lerch MM, Aghdassi AA, Grote T, Heuser DJ, Ignatavicius P, Malecka-Panas E, Domínguez-Muñoz JE, López-Serrano A, Auriemma F, Oracz G, Duman D, Gubergrits N (2024)

Publication Type: Journal article

Publication year: 2024


DOI: 10.1016/j.cgh.2023.12.010


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.

Authors with CRIS profile

Involved external institutions

Erasmus University Medical Center (MC) NL Netherlands (NL) Aalborg Universitetshospital DK Denmark (DK) Ospedale San Raffaele (früher: Centro San Raffaele del Monte Tabor Foundation) IT Italy (IT) Akershus University Hospital / Akershus universitetssykehus (Ahus) NO Norway (NO) The University of Liverpool GB United Kingdom (GB) University of Pécs / Pécsi Tudományegyetem HU Hungary (HU) University of Sheffield GB United Kingdom (GB) Newcastle upon Tyne Hospitals NHS Foundation Trust GB United Kingdom (GB) Philipps-Universität Marburg DE Germany (DE) Universitätsklinikum Mannheim DE Germany (DE) Masaryk University CZ Czech Republic (CZ) I.M. Sechenov First Moscow State Medical University RU Russian Federation (RU) GBUZ Moscow Clinical Scientific Center RU Russian Federation (RU) University of Oxford GB United Kingdom (GB) Institute for Clinical and Experimental Medicine (IKEM) CZ Czech Republic (CZ) Marmara University TR Turkey (TR) University of Amsterdam NL Netherlands (NL) Universitätsklinikum Ulm DE Germany (DE) Technische Universität München (TUM) DE Germany (DE) Beaujon Hospital / Hôpital Beaujon FR France (FR) Klinikum der Universität München DE Germany (DE) Universität Leipzig DE Germany (DE) Universitätsmedizin Greifswald / Universitätsklinikum Greifswald DE Germany (DE) Humanitas Mater Domini IT Italy (IT) University Hospital Olomouc / Fakultní Nemocnice Olomouc CZ Czech Republic (CZ) University Hospital Brno CZ Czech Republic (CZ) Acıbadem Mehmet Ali Aydınlar University / Acıbadem Mehmet Ali Aydınlar Üniversitesi TR Turkey (TR) Istanbul University / İstanbul Üniversitesi TR Turkey (TR) Università Vita-Salute San Raffaele (UniSR) IT Italy (IT) Universitätsklinikum Schleswig-Holstein (UKSH) DE Germany (DE) Mediterranean Institute for Transplantation and Advanced Specialized Therapies / Istituto Mediterraneo per i Trapianti e Terapie ad Alta Specializzazione (ISMETT) IT Italy (IT) Biocruces Health Research Institute ES Spain (ES) Martin-Luther-Universität Halle-Wittenberg (MLU) DE Germany (DE) Karolinska University Hospital / Karolinska Universitetssjukhuset SE Sweden (SE) Universitätsklinikum Essen DE Germany (DE) University Medical Centre Utrecht (UMC Utrecht) NL Netherlands (NL)

How to cite


Overbeek, K.A., Poulsen, J.L., Lanzillotta, M., Vinge-Holmquist, O., Macinga, P., Demirci, A.F.,... Gubergrits, N. (2024). Type 1 Autoimmune Pancreatitis in Europe: Clinical Profile and Response to Treatment. Clinical Gastroenterology and Hepatology. https://doi.org/10.1016/j.cgh.2023.12.010


Overbeek, Kasper A., et al. "Type 1 Autoimmune Pancreatitis in Europe: Clinical Profile and Response to Treatment." Clinical Gastroenterology and Hepatology (2024).

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