Publication details

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

Authors	OVERBEEK Kasper A POULSEN Jakob L LANZILLOTTA Marco VINGE-HOLMQUIST Olof MACINGA Peter DEMIRCI A Fatih SINDHUNATA Daniko P BACKHUS Johanna ALGÜL Hana BUIJS Jorie LEVY Philippe KIRIUKOVA Mariia GONI Elisabetta HOLLENBACH Marcus MIKSCH Rainer C KUNOVSKÝ Lumír VUJASINOVIC Miroslav NIKOLIC Sara DICKERSON Luke HIRTH Michael NEURATH Markus F ZUMBLICK Malte VILA Josephine JALAL Mustafa BEYER Georg FROST Fabian CARRARA Silvia KALA Zdeněk JABANDŽIEV Petr SISMAN Gurhan AKYUZ Filiz CAPURSO Gabriele FALCONI Massimo ARLT Alexander VLEGGAAR Frank P BARRESI Luca GREENHALF Bill CZAKÓ László HEGYI Peter HOPPER Andrew NAYAR Manu K GRESS Thomas M VITALI Francesco SCHNEIDER Alexander HALLORAN Chris M TRNA Jan OKHLOBYSTIN Alexey V DAGNA Lorenzo CAHEN Djuna L BORDIN Dmitry REBOURS Vinciane MAYERLE Julia KAHRAMAN Alisan RASCH Sebastian CULVER Emma KLEGER Alexander MARTÍNEZ-MONEO Emma ROKKE Ola HUCL Tomas OLESEN Soren S BRUNO Marco J DELLA-TORRE Emanuel BEUERS Ulrich LÖHR J-Matthias ROSENDAHL Jonas
Year of publication	2024
Type	Article in Periodical
Magazine / Source	Clinical Gastroenterology and Hepatology
MU Faculty or unit	Faculty of Medicine
Citation
Web	https://www.sciencedirect.com/science/article/pii/S154235652301042X?via%3Dihub
Doi	http://dx.doi.org/10.1016/j.cgh.2023.12.010
Keywords	Autoimmune Pancreatitis; IgG4-Related Disease; IgG4-Related Pancreatitis
Description	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.