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

Research output: Contribution to journalResearch articleContributedpeer-review

Contributors

  • PrescrAIP Study Group - (Author)
  • Department of Internal Medicine I
  • Erasmus University Rotterdam
  • Aalborg University
  • Vita-Salute San Raffaele University
  • University of Oslo
  • St Olavs' University Hospital
  • Institute for Clinical and Experimental Medicine
  • Marmara University
  • University of Amsterdam
  • Ulm University
  • Technical University of Munich
  • Hopital Beaujon
  • GBUZ Moscow Clinical Scientific Center named after Loginov MHD
  • Ludwig Maximilian University of Munich
  • Leipzig University
  • University Hospital Olomouc
  • Masaryk University
  • Masaryk Memorial Cancer Institute
  • Karolinska Institutet
  • University of Liverpool (UOL)
  • Heidelberg University 
  • Friedrich-Alexander University Erlangen-Nürnberg
  • University of Marburg
  • Newcastle upon Tyne Hospitals NHS Foundation Trust
  • University of Sheffield
  • University of Greifswald
  • Humanitas Mater Domini
  • Acibadem Mehmet Ali Aydinlar Universitesi
  • Istanbul University
  • University Hospital Schleswig-Holstein Campus Kiel
  • University of Oldenburg
  • Utrecht University
  • Mediterranean Institute for Transplantation and Advanced Specialized Therapies
  • University of Pecs
  • Semmelweis University
  • Sechenov First Moscow State Medical University
  • University Hospital Carl Gustav Carus Dresden

Abstract

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.

Details

Original languageEnglish
Pages (from-to)994-1004.e10
JournalClinical gastroenterology and hepatology
Volume22
Issue number5
Publication statusPublished - May 2024
Peer-reviewedYes

External IDs

PubMed 38184096
ORCID /0000-0002-2421-6127/work/204617864

Keywords

ASJC Scopus subject areas

Keywords

  • Autoimmune Pancreatitis, IgG4-Related Disease, IgG4-Related Pancreatitis