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Pediatric Autoimmune Pancreatitis: Clinical Findings and Outcomes in Sweden
Miroslav Vujasinovic1,2, Fredrik Lindgren3,4, Nikolaos Kartalis3,5
1Department of Upper Abdominal Diseases, Karolinska University Hospital, Stockholm, Sweden.
Insights
Pediatric autoimmune pancreatitis (AIP) is rare, often involving multiple organs, especially inflammatory bowel disease (IBD). Glucocorticoid treatment leads to remission in most children with AIP and IBD.
Area of Science:
- Pediatric Gastroenterology
- Autoimmune Diseases
- Pancreatology
Background:
- Pediatric autoimmune pancreatitis (AIP) is a rare pancreatic disorder with unclear causes.
- It is a risk factor for chronic pancreatitis in children.
- This study examines a large Swedish cohort of pediatric AIP patients.
Purpose of the Study:
- To describe the demographics of pediatric AIP patients.
- To outline the clinical characteristics and outcomes of pediatric AIP.
- To analyze treatment responses and long-term prognosis in pediatric AIP.
Main Methods:
- Retrospective analysis of medical records for pediatric AIP patients.
- Data collected from January 2006 to December 2022.
- Inclusion criteria: diagnosis of pediatric AIP.
Main Results:
- Thirty-seven pediatric AIP patients (mean age 13.5 years) were analyzed.
- Most presented with mild acute pancreatitis (62.2%), multi-organ involvement (81.1%), and inflammatory bowel disease (IBD) (62.2%).
- Eighty-four percent of treated patients achieved remission with glucocorticoids; exocrine insufficiency decreased from 43.2% to 24.3%.
Conclusions:
- Pediatric AIP is rare and frequently co-occurs with IBD and multi-organ involvement.
- Glucocorticoid therapy is effective, leading to remission in most cases.
- Co-management of IBD may enhance outcomes for pediatric AIP patients.
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.
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Pancreatitis is the inflammation of the pancreas, which occurs when the immune system becomes active and causes swelling, pain, and disruptions in organ function. Pancreatitis can manifest as either an acute or chronic condition.
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