Related Experiment Videos
Autoimmune Pancreatitis in Children: Characteristic Features, Diagnosis, and Management
Isabelle Scheers1,2, Joseph J Palermo3, Steven Freedman4
1Hospital for Sick Children, Toronto, Ontario, Canada.
Insights
Pediatric autoimmune pancreatitis (AIP) presents uniquely, often mimicking adult type 2 AIP. This study details the largest cohort of children with AIP, aiding diagnosis and management.
Area of Science:
- Pediatric gastroenterology
- Immunology
- Hepatology
Background:
- Autoimmune pancreatitis (AIP) is increasingly recognized, but pediatric data are limited.
- Pediatric AIP may present and progress differently than in adults.
- Understanding pediatric AIP is crucial for accurate diagnosis and treatment.
Purpose of the Study:
- To determine the characteristic features of AIP in children.
- To characterize the clinical presentation, imaging findings, histology, and outcomes of pediatric AIP.
- To provide a comprehensive overview of the largest pediatric AIP cohort to date.
Main Methods:
- Systematic literature search for pediatric AIP cases.
- Analysis of data from the INSPPIRE database (international multicenter study).
- Review of cases from the Cliniques Universitaires St-Luc (CUSL) registry.
Main Results:
- 48 pediatric AIP cases were identified (30 literature, 14 INSPPIRE, 4 CUSL).
- Common symptoms included abdominal pain (91%) and obstructive jaundice (42%); elevated IgG4 was infrequent (22%).
- Imaging showed gland enlargement (83%), duct irregularity (64%), and bile duct strictures (55%); histology revealed inflammation and fibrosis. Children responded well to steroids, but exocrine/endocrine dysfunction occurred.
Conclusions:
- Pediatric AIP has a distinct presentation, resembling adult type 2 AIP.
- This study provides the largest dataset of pediatric AIP cases.
- Findings will aid in diagnosing and managing pediatric AIP and guide future research.
Objectives:
Autoimmune pancreatitis (AIP) is an increasingly recognized disease entity, but data in children are limited. AIP presentation and outcome in children might differ from the adult experience. We aim to determine the characteristic features of AIP in children.
Methods:
Data about clinical symptoms, imaging, histology, and treatment were collected using two sources: (i) a systematic literature search and (ii) the INSPPIRE database, the largest international multicenter study of pancreatitis in children and the Cliniques Universitaires St-Luc (CUSL) registry.
Results:
We identified 48 AIP cases: 30 from literature review, 14 from INSPPIRE, and 4 from CUSL. The median age at diagnosis was 13 years (range 2-17 years). Abdominal pain (43/47, 91%) and/or obstructive jaundice (20/47, 42%) were the most common symptoms at diagnosis. Elevated serum IgG4 levels were only observed in 9/40 (22%) children. Cross-sectional imaging studies were abnormal in all children including hypointense global or focal gland enlargement (39/47, 83%), main pancreatic duct irregularity (30/47, 64%), and common bile duct stricture (26/47, 55%). A combination of lymphoplasmacytic inflammation, pancreatic fibrosis, and ductal granulocyte infiltration were the main histological findings (18/25, 72%). Children with AIP had a prompt clinical response to steroids. Complications of AIP included failure of exocrine (4/25, 16%) and endocrine (3/27, 11%) pancreas function.
Conclusions:
Pediatric AIP has a distinct presentation with features similar to type 2 AIP in adults. This comprehensive report on the largest group of children with AIP to date is expected to help with the diagnosis and management of this disease and pave the way for future research studies.