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

Isabelle Scheers; Joseph J Palermo; Steven Freedman; Michael Wilschanski; Uzma Shah; Maisam Abu-El-Haija; Bradley Barth; Douglas S Fishman; Cheryl Gariepy; Matthew J Giefer; Melvin B Heyman; Ryan W Himes; Sohail Z Husain; Tom K Lin; Quin Liu; Mark Lowe; Maria Mascarenhas; Veronique Morinville; Chee Y Ooi; Emily R Perito; David A Piccoli; John F Pohl; Sarah J Schwarzenberg; David Troendle; Steven Werlin; Bridget Zimmerman; Aliye Uc; Tanja Gonska

doi:10.1038/ajg.2017.85

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

Journal article

Open access

Peer reviewed

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

Isabelle Scheers, Joseph J Palermo, Steven Freedman, Michael Wilschanski, Uzma Shah, Maisam Abu-El-Haija, Bradley Barth, Douglas S Fishman, Cheryl Gariepy, Matthew J Giefer, …

The American journal of gastroenterology, Vol.112(10), pp.1604-1611

10/2017

DOI: 10.1038/ajg.2017.85

PMCID: PMC5908471

PMID: 28374818

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https://www.ncbi.nlm.nih.gov/pmc/articles/5908471View

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Abstract

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. 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. 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. 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.

Glucocorticoids - therapeutic use

Immunoglobulin G - blood

Humans

Pancreatitis, Chronic - diagnosis

Child, Preschool

Male

Jaundice, Obstructive - etiology

Pancreatic Function Tests - methods

Registries - statistics & numerical data

Child

Pancreas - diagnostic imaging

Pancreatitis, Chronic - immunology

Abdominal Pain - diagnosis

Diagnosis, Differential

Abdominal Pain - etiology

Autoimmune Diseases - complications

International Cooperation

Autoimmune Diseases - diagnosis

Pancreas - immunology

Autoimmune Diseases - blood

Pancreatitis, Chronic - complications

Adolescent

Pancreatitis, Chronic - therapy

Autoimmune Diseases - therapy

Jaundice, Obstructive - diagnosis

Disease Management

Details

Title: Subtitle: Autoimmune Pancreatitis in Children: Characteristic Features, Diagnosis, and Management
Creators: Isabelle Scheers - Cliniques Universitaires St-Luc, Brussels, Belgium
Joseph J Palermo - Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA
Steven Freedman - Harvard Medical School, Beth Israel Deaconess Medical Center, Boston, Massachusetts, USA
Michael Wilschanski - Hadassah Hebrew University Hospital, Jerusalem, Israel
Uzma Shah - Harvard Medical School, Massachusetts General Hospital for Children, Boston, Massachusetts, USA
Maisam Abu-El-Haija - Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA
Bradley Barth - University of Texas Southwestern Medical School, Dallas, Texas, USA
Douglas S Fishman - Baylor College of Medicine, Houston, Texas, USA
Cheryl Gariepy - Nationwide Children's Hospital, Columbus, Ohio, USA
Matthew J Giefer - Seattle Children's Hospital, Seattle, Washington, USA
Melvin B Heyman - University of California at San Francisco, San Francisco, California, USA
Ryan W Himes - Baylor College of Medicine, Houston, Texas, USA
Sohail Z Husain - Children's Hospital of Pittsburgh of UPMC, Pittsburgh, Pennsylvania, USA
Tom K Lin - Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA
Quin Liu - Keck School of Medicine, University of Southern California, Children's Hospital Los Angeles, Los Angeles, California, USA
Mark Lowe - Children's Hospital of Pittsburgh of UPMC, Pittsburgh, Pennsylvania, USA
Maria Mascarenhas - The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA
Veronique Morinville - Montreal Children's Hospital, McGill University, Montreal, Quebec, Canada
Chee Y Ooi - Discipline of Paediatrics, School of Women's and Children's Health, Medicine, University of New South Wales and Sydney Children's Hospital Randwick, Sydney, New South Wales, Australia
Emily R Perito - University of California at San Francisco, San Francisco, California, USA
David A Piccoli - The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA
John F Pohl - University of Utah, Salt Lake City, Utah, USA
Sarah J Schwarzenberg - University of Minnesota Masonic Children's Hospital, Minneapolis, Minnesota, USA
David Troendle - University of Texas Southwestern Medical School, Dallas, Texas, USA
Steven Werlin - Medical College of Wisconsin, Milwaukee, Wisconsin, USA
Bridget Zimmerman - University of Iowa Carver College of Medicine, Iowa City, Iowa, USA
Aliye Uc - University of Iowa Carver College of Medicine, Iowa City, Iowa, USA
Tanja Gonska - Hospital for Sick Children, Toronto, Ontario, Canada
Resource Type: Journal article
Publication Details: The American journal of gastroenterology, Vol.112(10), pp.1604-1611
DOI: 10.1038/ajg.2017.85
PMID: 28374818
PMCID: PMC5908471
NLM abbreviation: Am J Gastroenterol
ISSN: 0002-9270
eISSN: 1572-0241
Publisher: United States
Grant note: K23 DK099253 / NIDDK NIH HHS U01 DK108326 / NIDDK NIH HHS P30 DK078392 / NIDDK NIH HHS R21 DK096327 / NIDDK NIH HHS U01 DK108334 / NIDDK NIH HHS UL1 TR000442 / NCATS NIH HHS P30 DK054759 / NIDDK NIH HHS
Language: English
Date published: 10/2017
Academic Unit: Stead Family Department of Pediatrics; Biostatistics; Radiation Oncology; Gastroenterology, Hepatology, Pancreatology, and Nutrition
Record Identifier: 9983997321502771

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