Maintenance Treatment for Type 1 Autoimmune Pancreatitis: Effectiveness and Development of the PrescrAIP Relapse Prediction Model

Marco Lanzillotta ¹, Peter Macinga ², Jakob L Poulsen ³

¹Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), IRCCS San Raffaele Scientific Institute, Milan, Italy; Università Vita-Salute San Raffaele, Milan, Italy.
²Department of Gastroenterology and Hepatology, Institute for Clinical and Experimental Medicine, Prague, Czech Republic.
³Centre for Pancreatic Diseases, Department of Gastroenterology and Hepatology, Aalborg University Hospital, Aalborg, Denmark.
⁴Institute of Clinical Medicine, University of Oslo, Oslo, Norway; Department of Digestive Surgery, St Olavs Hospital, Trondheim University Hospital, Trondheim, Norway.
⁵Department of Internal Medicine, Marmara University Research and Education Hospital, Istanbul, Turkey.
⁶Pancreato-Biliary Endoscopy and Endosonography Division, Pancreas Translational and Clinical Research Center, IRCCS San Raffaele Scientific Institute, Milan, Italy.
⁷Department of Internal Medicine I, University Hospital Ulm, Ulm, Germany.
⁸Comprehensive Cancer Center Munich TUM, Institute for Tumor Metabolism, Technical University of Munich, Klinikum rechts der Isar, Munich, Germany.
⁹Pancreatology and Digestive Oncology Department, APHP Beaujon Hospital, Université Paris-Cité, Clichy, France.
¹⁰Department of Upper Gastrointestinal, Pancreatic, and Biliary Diseases, A.S. Loginov Moscow Clinical Research Center, Moscow, Russia.
¹¹Department of Medicine II, University Hospital, Ludwig Maximilian University Munich, Munich, Germany.
¹²Division of Gastroenterology, Medical Department II - Oncology, Gastroenterology, Hepatology, Pulmonology, Infectious Diseases, University of Leipzig Medical Center, Leipzig, Germany.
¹³Department of General, Visceral, and Transplantation Surgery, University Hospital, Ludwig Maximilian University Munich, Munich, Germany; Department of Orthopaedics and Trauma Surgery, Musculoskeletal University Center Munich (MUM), Universitiy Hospital, Ludwig Maximilian University Munich, Munich, Germany.
¹⁴Second Department of Internal Medicine - Gastroenterology and Geriatrics, University Hospital Olomouc, Faculty of Medicine and Dentistry, Palacky University Olomouc, Olomouc, Czech Republic; Department of Surgery, University Hospital Brno, Faculty of Medicine, Masaryk University, Brno, Czech Republic; Department of Gastroenterology and Digestive Endoscopy, Masaryk Memorial Cancer Institute, Brno, Czech Republic.
¹⁵Department of Upper Abdominal Diseases, Karolinska University Hospital, Stockholm, Sweden.
¹⁶Department of Upper Abdominal Diseases, Karolinska University Hospital, Stockholm, Sweden; Department of Gastroenterology, University Medical Center Maribor, Maribor, Slovenia.
¹⁷Department of Molecular and Clinical Cancer Medicine, Institute of Translational Medicine, University of Liverpool, Liverpool, United Kingdom.
¹⁸Department of Medicine II, University Medical Center Mannheim, Medical Faculty at Mannheim, University of Heidelberg, Mannheim, Germany.
¹⁹Department of Medicine I, Deutsches Zentrum Immuntherapie (DZI), University Erlangen-Nürnberg, Erlangen, Germany.
²⁰Internal Medicine SP Gastroenterology, Endocrinology and Infectiology, University Hospital Marburg, Philipps University Marburg, Marburg, Germany.
²¹HPB Unit, Freeman Hospital, Newcastle Upon Tyne, United Kingdom.
²²Department of Infection, Immunity and Cardiovascular Disease, University of Sheffield, Sheffield, United Kingdom.
²³Department of Medicine A, University Medicine Greifswald, Greifswald, Germany.
²⁴Department of Biomedical Sciences, Humanitas University, Milan, Italy; IRCCS Humanitas Research Hospital, Milan, Italy.
²⁵Department of Surgery, University Hospital Brno, Faculty of Medicine, Masaryk University, Brno, Czech Republic.
²⁶Department of Pediatrics, University Hospital Brno, Faculty of Medicine, Masaryk University, Brno, Czech Republic; Central European Institute of Technology, Masaryk University, Brno, Czech Republic.
²⁷Acibadem Mehmet Ali Aydinlar University School of Medicine, Istanbul, Turkey.
²⁸Department of Gastroenterology, Istanbul University, Istanbul Faculty of Medicine, Istanbul, Turkey.
²⁹Università Vita-Salute San Raffaele, Milan, Italy; Pancreato-Biliary Endoscopy and Endosonography Division, Pancreas Translational and Clinical Research Center, IRCCS San Raffaele Scientific Institute, Milan, Italy.
³⁰Università Vita-Salute San Raffaele, Milan, Italy; Division of Pancreatic and Transplant Surgery, Pancreas Translational and Clinical Research Center, San Raffaele Scientific Institute IRCCS, Vita-Salute San Raffaele University, Milan, Italy.
³¹Medizinische Klinik, Israelitisches Krankenhaus Hamburg, Hamburg, Germany.
³²Department of Gastroenterology and Hepatology, Tytgat Institute for Liver and Intestinal Research, AGEM, Amsterdam University Medical Center, Amsterdam, The Netherlands.
³³Pancreas Unit, Department of Diagnostic and Therapeutic Services, Mediterranean Institute for Transplantation and Advanced Specialized Therapies (IRCSS-ISMETT), Palermo, Italy.
³⁴Center of Gastroenterology, Department of Medicine, University of Szeged, Szeged, Hungary.
³⁵Institute of Pancreatic Diseases and Centre for Translational Medicine, Semmelweis University, Budapest, Hungary; Translational Pancreatology Research Group, Interdisciplinary Centre of Excellence for Research Development and Innovation, University of Szeged, Szeged, Hungary.
³⁶HPB Unit, Freeman Hospital, Newcastle Upon Tyne, United Kingdom; Population and Health Sciences Institute, Newcastle University, Newcastle upon Tyne, United Kingdom.
³⁷Department of General Internal Medicine, University Hospital Brno and Faculty of Medicine, Masaryk University, Brno, Czech Republic.
³⁸I.M. Sechenov First Moscow State Medical University, Moscow, Russia.
³⁹Department of Gastroenterology & Hepatology, Erasmus MC, University Medical Center, Rotterdam, The Netherlands.
⁴⁰Department of Upper Gastrointestinal, Pancreatic, and Biliary Diseases, A.S. Loginov Moscow Clinical Research Center, Moscow, Russia; Department of Outpatient Therapy and Family Medicine, Tver State Medical University, Tver, Russia.
⁴¹Division of Gastroenterology and Hepatology, Department of Internal Medicine, Essen University Hospital, University of Duisberg-Essen, Essen, Germany; Unique Medicine, Lichtentaler Str. 27, 76530 Baden-Baden, Germany.
⁴²Translational Gastroenterology Unit, John Radcliffe Hospital and Nuffield Department of Medicine, University of Oxford, Oxford, United Kingdom.
⁴³Institute of Molecular Oncology and Stem Cell Biology, Ulm University Hospital, Ulm, Germany; Division of Interdisciplinary Pancreatology, Department of Internal Medicine I, Ulm University Hospital, Ulm, Germany.
⁴⁴Biocruces, Grupo Transplante Hepático, Osakidetza, Hospital Universitario Cruces, Servicio Aparato Digestivo, Barakaldo, Spain.
⁴⁵Department of Internal Medicine I, Martin Luther University, Halle (Saale), Germany.

⁰Unit of Immunology, Rheumatology, Allergy and Rare Diseases (UnIRAR), IRCCS San Raffaele Scientific Institute, Milan, Italy; Università Vita-Salute San Raffaele, Milan, Italy.

Clinical Gastroenterology and Hepatology : the Official Clinical Practice Journal of the American Gastroenterological Association +

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September 15, 2025

View abstract on PubMed

Summary

Maintenance therapy for type 1 autoimmune pancreatitis (AIP) reduces relapse risk, particularly in high-risk patients. The new PrescrAIP score helps personalize treatment decisions for AIP relapse prevention.

Area Of Science

Gastroenterology
Immunology
Clinical Medicine

Background

Type 1 autoimmune pancreatitis (AIP) is a chronic relapsing-remitting inflammatory condition.
Management of AIP often involves multiple treatment courses due to frequent relapses.

Purpose Of The Study

To evaluate the effectiveness of maintenance treatment in preventing AIP relapse.
To develop the PrescrAIP risk score for predicting relapse risk and maintenance treatment benefit.

Main Methods

Retrospective analysis of 577 patients diagnosed with type 1 AIP.
Development of a multivariable prediction model (PrescrAIP score) using Cox regression.
Internal and internal-external validation of the predictive model and nomogram.

Main Results

The 3-year relapse risk was 28%, significantly lower with maintenance treatment (22% vs. 35%).
The PrescrAIP score incorporates factors like maintenance treatment, sex, and clinical presentation.
Maintenance treatment benefit was observed in patients with a PrescrAIP score >155.

Conclusions

Maintenance therapy is effective in reducing relapse in high-risk type 1 AIP patients.
The PrescrAIP score may guide personalized maintenance treatment strategies for AIP.

Keywords:

Autoimmune Pancreatitis IgG4 Multicenter Study Personalized Treatment Prediction Model

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