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Maintenance treatment for type 1 autoimmune pancreatitis: effectiveness and development of the PrescrAIP relapse prediction model

Background & Aims - Type 1 autoimmune pancreatitis (AIP) is a relapsing remitting disorder that often requires multiple treatment courses. Our aims were to assess the efficacy of maintenance treatment in preventing relapse and to develop the PrescrAIP risk score predicting relapse risk and the b...

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Main Authors: Lanzillotta, Marco (Author) , Macinga, Peter (Author) , Poulsen, Jakob L. (Author) , Vinge-Holmquist, Olof (Author) , Demirci, A. Fatih (Author) , Tacelli, Matteo (Author) , Backhus, Johanna (Author) , Algül, Hana (Author) , Descourvieres, Clémence (Author) , Kiriukova, Mariia (Author) , Goni, Elisabetta (Author) , Hollenbach, Marcus (Author) , Miksch, Rainer C. (Author) , Kunovsky, Lumir (Author) , Vujasinovic, Miroslav (Author) , Nikolic, Sara (Author) , Dickerson, Luke (Author) , Hirth, Michael (Author) , Neurath, Markus F. (Author) , Zumblick, Malte (Author) , Vila, Josephine (Author) , Jalal, Mustafa (Author) , Beyer, Georg (Author) , Frost, Fabian (Author) , Carrara, Silvia (Author) , Kala, Zdenek (Author) , Jabandziev, Petr (Author) , Sisman, Gurhan (Author) , Akyuz, Filiz (Author) , Capurso, Gabriele (Author) , Falconi, Massimo (Author) , Arlt, Alexander (Author) , Vleggaar, Frank P. (Author) , Barresi, Luca (Author) , Greenhalf, Bill (Author) , Czakó, László (Author) , Hegyi, Peter (Author) , Hopper, Andrew (Author) , Nayar, Manu K. (Author) , Gress, Thomas M. (Author) , Vitali, Francesco (Author) , Schneider, Alexander (Author) , Halloran, Chris M. (Author) , Trna, Jan (Author) , Okhlobystin, Alexey V. (Author) , Dagna, Lorenzo (Author) , Cahen, Djuna L. (Author) , Bordin, Dmitry (Author) , Rebours, Vinciane (Author) , Mayerle, Julia (Author) , Kahraman, Alisan (Author) , Rasch, Sebastian (Author) , Culver, Emma (Author) , Kleger, Alexander (Author) , Martínez-Moneo, Emma (Author) , Hucl, Tomas (Author) , Olesen, Søren S. (Author) , Bruno, Marco J. (Author) , Beuers, Ulrich (Author) , Della-Torre, Emanuel (Author) , Löhr, J. Matthias (Author) , Rosendahl, Jonas (Author) , Overbeek, Kasper A. (Author)
Format: Article (Journal)
Language:English
Published: May 2026
In: Clinical gastroenterology and hepatology
Year: 2026, Volume: 24, Issue: 5, Pages: 1321-1331
ISSN:1542-7714
DOI:10.1016/j.cgh.2025.09.005
Online Access:Verlag, lizenzpflichtig, Volltext: https://doi.org/10.1016/j.cgh.2025.09.005
Verlag, lizenzpflichtig, Volltext: https://www.sciencedirect.com/science/article/pii/S1542356525007955
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Author Notes:Marco Lanzillotta, Peter Macinga, Jakob L. Poulsen, Olof Vinge-Holmquist, A. Fatih Demirci, Matteo Tacelli, Johanna Backhus, Hana Algül, Clémence Descourvieres, Mariia Kiriukova, Elisabetta Goni, Marcus Hollenbach, Rainer C. Miksch, Lumir Kunovsky, Miroslav Vujasinovic, Sara Nikolic, Luke Dickerson, Michael Hirth, Markus F. Neurath, Malte Zumblick, Josephine Vila, Mustafa Jalal, Georg Beyer, Fabian Frost, Silvia Carrara, Zdenek Kala, Petr Jabandziev, Gurhan Sisman, Filiz Akyuz, Gabriele Capurso, Massimo Falconi, Alexander Arlt, Frank P. Vleggaar, Luca Barresi, Bill Greenhalf, László Czakó, Peter Hegyi, Andrew Hopper, Manu K. Nayar, Thomas M. Gress, Francesco Vitali, Alexander Schneider, Chris M. Halloran, Jan Trna, Alexey V. Okhlobystin, Lorenzo Dagna, Djuna L. Cahen, Dmitry Bordin, Vinciane Rebours, Julia Mayerle, Alisan Kahraman, Sebastian Rasch, Emma Culver, Alexander Kleger, Emma Martínez-Moneo, Tomas Hucl, Søren S. Olesen, Marco J. Bruno, Ulrich Beuers, Emanuel Della-Torre, J. Matthias Löhr, Jonas Rosendahl, and Kasper A. Overbeek, on behalf of the PrescrAIP Study Group
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Summary:Background & Aims - Type 1 autoimmune pancreatitis (AIP) is a relapsing remitting disorder that often requires multiple treatment courses. Our aims were to assess the efficacy of maintenance treatment in preventing relapse and to develop the PrescrAIP risk score predicting relapse risk and the benefit of maintenance treatment. - Methods - We retrospectively analyzed patients meeting international diagnostic criteria for type 1 AIP who reached partial or complete remission after initial treatment. The primary outcome was disease relapse, defined as recurrence of symptoms and/or radiologic findings. We developed a multivariable prediction model using Cox proportional hazards regression, performed internal and internal-external validation, and built a predictive nomogram. - Results - We included 577 patients (68% male). During a median follow-up of 34 months (interquartile range, 13-69 months), we observed 154 relapses. The overall 3-year relapse risk was 28% (95% confidence interval [CI], 24%-32%), lower in patients receiving maintenance treatment than in those without (22% vs 35%; P < .001). The final PrescrAIP model incorporated protective factors (maintenance treatment, prior surgery, focal mass, female sex) and risk factors (biliary involvement, other organ involvement, IgG4 elevation, allergy, jaundice, acute pancreatitis). The model showed moderate discrimination (Concordance index, 0.69) and good calibration. Internal-external validation yielded Concordance index values ranging from 0.64 to 0.71. Maintenance treatment significantly reduced relapse in patients with a PrescrAIP score >155, but not in those with lower scores. - Conclusions - Maintenance therapy reduced relapse only in patients at high relapse risk. Once externally validated, the PrescrAIP score may guide personalized maintenance treatment decisions.
Item Description:Online verfügbar: 13. September 2025, Artikelversion: 21. April 2026
Gesehen am 07.07.2026
Physical Description:Online Resource
ISSN:1542-7714
DOI:10.1016/j.cgh.2025.09.005

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