Pediatric Autoimmune Pancreatitis: Clinical Findings and Outcomes in Sweden

Abstract

Introduction: Pediatric autoimmune pancreatitis (AIP) is a rare form of pancreatitis in children with poorly understood pathophysiology. It is a recognized risk factor for chronic pancreatitis in approximately 4% of pediatric cases. This study aims to describe the demographics, clinical characteristics, and outcomes of a large Swedish cohort of pediatric AIP patients. Patients and Methods: A retrospective analysis of medical records was conducted for patients diagnosed with pediatric AIP between January 2006 and December 2022. Results: Thirty-seven patients were included (20 males, 17 females; mean age 13.5 ± 2.8 years). None had a family history of pancreatic diseases. Most patients (62.2%) presented with acute pancreatitis, followed by weight loss (45.9%), abdominal pain (43.2%), jaundice (21.6%), and fatigue (16.2%). Acute pancreatitis was mild in all cases according to the Atlanta criteria. Multi-organ involvement was observed in 81.1% of patients, and inflammatory bowel disease (IBD) was present in 62.2%. A total of 75.7% of patients received treatment, mainly glucocorticoids, while 24.3% had spontaneous regression. Complete clinical and radiological remission was achieved in 83.8% of treated patients. Pancreatic exocrine insufficiency was present in 43.2% of patients at diagnosis, reducing to 24.3% at the final follow-up. No patients developed diabetes mellitus, except for one who underwent total pancreatectomy due to suspected pancreatic tumor. Conclusions: Pediatric AIP is a rare condition often associated with multi-organ involvement particularly IBD. Most patients respond well to glucocorticoid treatment and achieve remission. Managing IBD may improve the outcomes for both conditions.