Luca Barresi1, Matteo Tacelli1,2, Stefano Francesco Crinò3, Fabia Attili4, Maria Chiara Petrone5, Germana De Nucci6, Silvia Carrara7, Guido Manfredi8, Gabriele Capurso5,9, Claudio Giovanni De Angelis10, Lucia Crocellà11, Alberto Fantin12, Maria Francesca Dore13, Alessandra Tina Garribba14, Ilaria Tarantino1, Nicolò De Pretis15, Danilo Pagliari16, Gemma Rossi5, Gianpiero Manes6, Paoletta Preatoni7, Ilenia Barbuscio1,12, Fabio Tuzzolino17, Mario Traina1, Luca Frulloni15, Guido Costamagna4,18, Paolo Giorgio Arcidiacono5, Elisabetta Buscarini8, Raffaele Pezzilli19. 1. Endoscopy Service, Department of Diagnostic and Therapeutic Services, IRCCS-ISMETT (Mediterranean Institute for Transplantation and Highly Specialized Therapies), Palermo, Italy. 2. Section of Gastroenterology, Biomedical Department of Internal and Specialized Medicine (DI.BI.M.I.S.), University of Palermo, Palermo, Italy. 3. Gastroenterology and Digestive Endoscopy Unit, The Pancreas Institute, University Hospital of Verona, Verona, Italy. 4. Digestive Endoscopy Unit, Fondazione Policlinico Universitario Agostino Gemelli IRCCS, Roma, Italy. 5. Pancreato-Biliary Endoscopy and Endosonography Division, Pancreas Translational and Clinical Research Center, San Raffaele Scientific Institute IRCCS, Vita-Salute San Raffaele University, Milano, Italy. 6. Gastroenterology and Digestive Endoscopy Unit, ASST Rhodense, Garbagnate Milanese Hospitals, Milano, Italy. 7. Digestive Endoscopy Unit, Division of Gastroenterology, Humanitas Clinical and Research Center-IRCCS Rozzano (MI), Italy. 8. Gastroenterology and Digestive Endoscopy Department, Maggiore Hospital, ASST Crema, Crema, Italy. 9. Digestive and Liver Disease Unit, S. Andrea Hospital, Roma, Italy. 10. Department of Medical Sciences, Division of Gastroenterology, University of Torino, Torino, Italy. 11. Gastroenterology Unit, Mauriziano Umberto I Hospital, Torino, Italy. 12. Department of Surgical, Oncological and Gastroenterological Sciences, Gastroenterology Division, Azienda Ospedaliera di Padova, University of Padova, Padua, Italy. 13. Gastroenterology Unit, Brotzu Hospital, Cagliari, Italy. 14. Gastroenterology Unit, AUSL Romagna, Ravenna, Italy. 15. Gastroenterology unit, Pancreas center, University of Verona, Verona, Italy. 16. Division of Internal Medicine and Gastroenterology & Pancreatic Unit, Fondazione Policlinico Universitario A. Gemelli-IRCCS, Universita' del Sacro Cuore, Roma, Italy. 17. Research Office, IRCCS ISMETT (Mediterranean Institute for Transplantation and Advanced Specialized Therapies), Palermo, Italy. 18. Centre for Endoscopic Research Therapeutics and Training-CERTT, Università del SacroCuore, Roma, Italy. 19. Pancreas Unit, Department of Gastroenterology, Sant'Orsola Polyclinic, Bologna, Italy.
Abstract
BACKGROUND: Autoimmune pancreatitis (AIP) is a rare, and relatively new, form of chronic pancreatitis. The management of AIP can vary considerably among different centres in daily clinical practice. OBJECTIVES: The aim of this study is to present a picture of epidemiological, clinical characteristics, outcomes, and the real-life practice in terms of management in several academic and non-academic centres in Italy. METHODS: Data on the clinical presentation, diagnostic work-up, treatments, frequency of relapses, and long-term outcomes were retrospectively collected in a cohort of AIP patients diagnosed at 14 centres in Italy. RESULTS: One hundred and six patients were classified as type 1 AIP, 48 as type 2 AIP, and 19 as not otherwise specified. Epidemiological, clinical, radiological, and serological characteristics, and relapses were similar to those previously reported for different types of AIP. Endoscopic cytohistology was available in 46.2% of cases, and diagnostic for AIP in only 35.2%. Steroid trial to aid diagnosis was administered in 43.3% cases, and effective in 93.3%. Steroid therapy was used in 70.5% of cases, and effective in 92.6% of patients. Maintenance therapy with low dose of steroid (MST) was prescribed in 25.4% of cases at a mean dose of 5 (±1.4) mg/die, and median time of MST was 60 days. Immunosuppressive drugs were rarely used (10.9%), and rituximab in 1.7%. Faecal elastase-1 was evaluated in only 31.2% of patients, and was pathological in 59.2%. CONCLUSIONS: In this cohort of AIP patients, diagnosis and classification for subtype was frequently possible, confirming the different characteristics of AIP1 and AIP2 previously reported. Nevertheless, we observed a low use of histology and steroid trial for a diagnosis of AIP. Steroid treatment was the most used therapy in our cohort. Immunosuppressants and rituximab were rarely used. The evaluation of exocrine pancreatic insufficiency is underemployed considering its high prevalence.
BACKGROUND:Autoimmune pancreatitis (AIP) is a rare, and relatively new, form of chronic pancreatitis. The management of AIP can vary considerably among different centres in daily clinical practice. OBJECTIVES: The aim of this study is to present a picture of epidemiological, clinical characteristics, outcomes, and the real-life practice in terms of management in several academic and non-academic centres in Italy. METHODS: Data on the clinical presentation, diagnostic work-up, treatments, frequency of relapses, and long-term outcomes were retrospectively collected in a cohort of AIP patients diagnosed at 14 centres in Italy. RESULTS: One hundred and six patients were classified as type 1 AIP, 48 as type 2 AIP, and 19 as not otherwise specified. Epidemiological, clinical, radiological, and serological characteristics, and relapses were similar to those previously reported for different types of AIP. Endoscopic cytohistology was available in 46.2% of cases, and diagnostic for AIP in only 35.2%. Steroid trial to aid diagnosis was administered in 43.3% cases, and effective in 93.3%. Steroid therapy was used in 70.5% of cases, and effective in 92.6% of patients. Maintenance therapy with low dose of steroid (MST) was prescribed in 25.4% of cases at a mean dose of 5 (±1.4) mg/die, and median time of MST was 60 days. Immunosuppressive drugs were rarely used (10.9%), and rituximab in 1.7%. Faecal elastase-1 was evaluated in only 31.2% of patients, and was pathological in 59.2%. CONCLUSIONS: In this cohort of AIP patients, diagnosis and classification for subtype was frequently possible, confirming the different characteristics of AIP1 and AIP2 previously reported. Nevertheless, we observed a low use of histology and steroid trial for a diagnosis of AIP. Steroid treatment was the most used therapy in our cohort. Immunosuppressants and rituximab were rarely used. The evaluation of exocrine pancreatic insufficiency is underemployed considering its high prevalence.
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Authors: Sara Nikolic; Marco Lanzillotta; Nikola Panic; Torkel B Brismar; Carlos Fernández Moro; Gabriele Capurso; Emanuel Della Torre; J-Matthias Löhr; Miroslav Vujasinovic Journal: United European Gastroenterol J Date: 2022-05-08 Impact factor: 6.866