Autoimmune Pancreatitis in Children: Characteristic Features, Diagnosis, and Management

Date:	2017 Oct
PMID:	28374818
Category:	2
Authors:	Isabelle Scheers 1 2, Joseph J Palermo 3, Steven Freedman 4, Michael Wilschanski 5, Uzma Shah 6, Maisam Abu-El-Haija 3, Bradley Barth 7, Douglas S Fishman 8, Cheryl Gariepy 9, Matthew J Giefer 10, Melvin B Heyman 11, Ryan W Himes 8, Sohail Z Husain 12, Tom K Lin 3, Quin Liu 13, Mark Lowe 12, Maria Mascarenhas 14, Veronique Morinville 15, Chee Y Ooi 16, Emily R Perito 11, David A Piccoli 14, John F Pohl 17, Sarah J Schwarzenberg 18, David Troendle 7, Steven Werlin 19, Bridget Zimmerman 20, Aliye Uc 20, Tanja Gonska 1
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.

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Acknowledgements:

The content of this article is solely the responsibility of the authors and does not necessarily represent the official views of the National Cancer Institute, the National Institute of Health, or the National Institute of Diabetes and Digestive and Kidney Diseases.

The Study of Chronic Pancreatitis, Diabetes, and Pancreatic Cancer (CPDPC) Research Consortia is supported and funded by grants from the National Cancer Institute and the National Institute of Diabetes and Digestive and Kidney Diseases under the following award numbers:

Project Number:	Awardee Organization
U01DK108326	Baylor College of Medicine
U01DK108314	Cedars-Sinai Medical Center
U01DK108332	Indiana University
U01DK108323	Kaiser Foundation Research Institute
U01DK108288	Mayo Clinic
U01DK108327	Ohio State University
U01DK108300	Stanford University
U01DK108320	University of Florida
U01DK108306	University of Pittsburgh
U01DK108328	University of Texas MD Anderson Cancer Center