TY - JOUR
T1 - Type 1 Autoimmune Pancreatitis in Europe
T2 - Clinical Profile and Response to Treatment
AU - Overbeek, Kasper A.
AU - Poulsen, Jakob L.
AU - PrescrAIP Study Group
AU - Lanzillotta, Marco
AU - Vinge-Holmquist, Olof
AU - Macinga, Peter
AU - Demirci, A. Fatih
AU - Sindhunata, Daniko P.
AU - Backhus, Johanna
AU - Algül, Hana
AU - Buijs, Jorie
AU - Levy, Philippe
AU - Kiriukova, Mariia
AU - Goni, Elisabetta
AU - Hollenbach, Marcus
AU - Miksch, Rainer C.
AU - Kunovsky, Lumir
AU - Vujasinovic, Miroslav
AU - Nikolic, Sara
AU - Dickerson, Luke
AU - Hirth, Michael
AU - Neurath, Markus F.
AU - Zumblick, Malte
AU - Vila, Josephine
AU - Jalal, Mustafa
AU - Beyer, Georg
AU - Frost, Fabian
AU - Carrara, Silvia
AU - Kala, Zdenek
AU - Jabandziev, Petr
AU - Sisman, Gurhan
AU - Akyuz, Filiz
AU - Capurso, Gabriele
AU - Falconi, Massimo
AU - Arlt, Alexander
AU - Vleggaar, Frank P.
AU - Barresi, Luca
AU - Greenhalf, Bill
AU - Czakó, László
AU - Hegyi, Peter
AU - Hopper, Andrew
AU - Nayar, Manu K.
AU - Gress, Thomas M.
AU - Vitali, Francesco
AU - Schneider, Alexander
AU - Halloran, Chris M.
AU - Trna, Jan
AU - Okhlobystin, Alexey V.
AU - Cahen, Djuna L.
AU - Bruno, Marco J.
AU - Haas, S. L.
N1 - Publisher Copyright: © 2024 The Author(s)
PY - 2024/5
Y1 - 2024/5
N2 - 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.
AB - 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.
UR - http://www.scopus.com/inward/record.url?scp=85184766789&partnerID=8YFLogxK
U2 - 10.1016/j.cgh.2023.12.010
DO - 10.1016/j.cgh.2023.12.010
M3 - Article
C2 - 38184096
AN - SCOPUS:85184766789
SN - 1542-3565
VL - 22
SP - 994-1004.e10
JO - Clinical Gastroenterology and Hepatology
JF - Clinical Gastroenterology and Hepatology
IS - 5
ER -