Faculty Collaboration Database

Autoimmune Pancreatitis in Children: Characteristic Features, Diagnosis, and Management. Am J Gastroenterol 2017 Oct;112(10):1604-1611

Date

04/05/2017

Pubmed ID

28374818

Pubmed Central ID

PMC5908471

DOI

10.1038/ajg.2017.85

Scopus ID

2-s2.0-85017109823 (requires institutional sign-in at Scopus site)   81 Citations

Abstract

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.

Author List

Scheers I, Palermo JJ, Freedman S, Wilschanski M, Shah U, Abu-El-Haija M, Barth B, Fishman DS, Gariepy C, Giefer MJ, Heyman MB, Himes RW, Husain SZ, Lin TK, Liu Q, Lowe M, Mascarenhas M, Morinville V, Ooi CY, Perito ER, Piccoli DA, Pohl JF, Schwarzenberg SJ, Troendle D, Werlin S, Zimmerman B, Uc A, Gonska T

Author

Steven L. Werlin MD Emeritus Professor in the Pediatrics department at Medical College of Wisconsin

MESH terms used to index this publication - Major topics in bold

Abdominal Pain
Adolescent
Autoimmune Diseases
Child
Child, Preschool
Diagnosis, Differential
Disease Management
Glucocorticoids
Humans
Immunoglobulin G
International Cooperation
Jaundice, Obstructive
Male
Pancreas
Pancreatic Function Tests
Pancreatitis, Chronic
Registries