Autoimmune Pancreatitis in Europe: Clinical Profile and Response to Treatment

Authors	OVERBEEK Kasper A POULSEN J LANZILLOTTA M VINGE-HOLMQUIST O MACINGA P DEMIRCI A SINDHUNATA D BACKHUS J ALGÜL H BUIJS J LEVY P KIRIUKOVA M GONI E HOLLENBACH M MIKSCH R KUNOVSKÝ Lumír VUJASINOVIC M NIKOLIC S DICKERSON L HIRTH M NEURATH M ZUMBLICK M VILA J JALAL M BEYER G FROST F CARRARA S KALA Zdeněk JABANDŽIEV Petr SISMAN G AKYUZ F CAPURSO G FALCONI M ARLT A VLEGGAAR F BARRESI L GREENHALF B CZAKÓ L HEGYI P HOPPER A NAYAR M GRESS T VITALI F SCHNEIDER A HALLORAN C TRNA Jan OKHLOBYSTIN A DAGNA L CAHEN D BORDIN D REBOURS V MAYERLE J KAHRAMAN A RASCH S CULVER E KLEGER A MARTÍNEZ-MONEO E ROKKE O HUCL T OLESEN S BRUNO M DELLA-TORRE E BEUERS U LÖHR J ROSENDAHL J
Year of publication	2022
Type	Conference abstract
MU Faculty or unit	Faculty of Medicine
Citation
Description	Introduction: Autoimmune pancreatitis (AIP) is a rare disease. Large-scale studies from Europe are lacking, and there is little evidence on the optimum treatment regimen. Purpose: To characterize AIP patients in a large pan-European cohort, study the effectiveness of current treatment regimens, and compare these to non-European cohorts. Materials and methods: We retrospectively analyzed all adults diagnosed since 2005 with AIP type 1 or not-otherwise-specified, who had not undergone pancreatic surgery, in 40 European university hospitals. Results: We screened 1079 individuals with suspected AIP, and included 735 for analysis (69% male; median age 57 years; 85% Caucasian). Rates of complete remission were 61% for those untreated (58/95) and 72% for steroid treatment (454/631). Rates of any remission (complete or partial) were 80% (76/95) for the untreated and 96% (603/631) for steroid-treated patients. Higher steroid doses were not more effective than lower doses (OR 0.428; 95%CI 0.054-3.387) and neither was a starting dose duration longer than two weeks (OR 0.908; 95%CI 0.818-1.009). Elevated IgG4 levels independently decreased the chance of complete remission (OR 0.639; 95%CI 0.427-0.955). Relapse occurred in 30% of patients. Relapses within 6 months of remission induction were independent of the steroid tapering duration, remission induction treatment duration, and total cumulative dose. Conclusions: European AIP patients display a slightly different demography, but the same presentation and course of disease as non-European populations. Their response to steroid treatment seems slightly lower. Patients with an elevated IgG4 level may need closer monitoring during remission induction. For remission induction, a starting dose of 0.4 mg/kg/day with a minimum of 20 mg for 2 weeks is an effective treatment regimen and there is no evidence to support more aggressive regimens.