title: Autoimmune Pancreatitis in Children: Characteristic Features, Diagnosis, and Management.
abstract: OBJECTIVE
  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.
authors.affiliation: Hospital for Sick Children, Toronto, Ontario, Canada.|Cliniques Universitaires St-Luc, Brussels, Belgium.
authors.forename: Isabelle
authors.initials: I
authors.lastname: Scheers
authors.affiliation: Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA.
authors.forename: Joseph J
authors.initials: JJ
authors.lastname: Palermo
authors.affiliation: Harvard Medical School, Beth Israel Deaconess Medical Center, Boston, Massachusetts, USA.
authors.forename: Steven
authors.initials: S
authors.lastname: Freedman
authors.affiliation: Hadassah Hebrew University Hospital, Jerusalem, Israel.
authors.forename: Michael
authors.initials: M
authors.lastname: Wilschanski
authors.affiliation: Harvard Medical School, Massachusetts General Hospital for Children, Boston, Massachusetts, USA.
authors.forename: Uzma
authors.initials: U
authors.lastname: Shah
authors.affiliation: Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA.
authors.forename: Maisam
authors.initials: M
authors.lastname: Abu-El-Haija
authors.affiliation: University of Texas Southwestern Medical School, Dallas, Texas, USA.
authors.forename: Bradley
authors.initials: B
authors.lastname: Barth
authors.affiliation: Baylor College of Medicine, Houston, Texas, USA.
authors.forename: Douglas S
authors.initials: DS
authors.lastname: Fishman
authors.affiliation: Nationwide Children's Hospital, Columbus, Ohio, USA.
authors.forename: Cheryl
authors.initials: C
authors.lastname: Gariepy
authors.affiliation: Seattle Children's Hospital, Seattle, Washington, USA.
authors.forename: Matthew J
authors.initials: MJ
authors.lastname: Giefer
authors.affiliation: University of California at San Francisco, San Francisco, California, USA.
authors.forename: Melvin B
authors.initials: MB
authors.lastname: Heyman
authors.affiliation: Baylor College of Medicine, Houston, Texas, USA.
authors.forename: Ryan W
authors.initials: RW
authors.lastname: Himes
authors.affiliation: Children's Hospital of Pittsburgh of UPMC, Pittsburgh, Pennsylvania, USA.
authors.forename: Sohail Z
authors.initials: SZ
authors.lastname: Husain
authors.affiliation: Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA.
authors.forename: Tom K
authors.initials: TK
authors.lastname: Lin
authors.affiliation: Keck School of Medicine, University of Southern California, Children's Hospital Los Angeles, Los Angeles, California, USA.
authors.forename: Quin
authors.initials: Q
authors.lastname: Liu
authors.affiliation: Children's Hospital of Pittsburgh of UPMC, Pittsburgh, Pennsylvania, USA.
authors.forename: Mark
authors.initials: M
authors.lastname: Lowe
authors.affiliation: The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA.
authors.forename: Maria
authors.initials: M
authors.lastname: Mascarenhas
authors.affiliation: Montreal Children's Hospital, McGill University, Montreal, Quebec, Canada.
authors.forename: Veronique
authors.initials: V
authors.lastname: Morinville
authors.affiliation: 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.
authors.forename: Chee Y
authors.initials: CY
authors.lastname: Ooi
authors.affiliation: University of California at San Francisco, San Francisco, California, USA.
authors.forename: Emily R
authors.initials: ER
authors.lastname: Perito
authors.affiliation: The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA.
authors.forename: David A
authors.initials: DA
authors.lastname: Piccoli
authors.affiliation: University of Utah, Salt Lake City, Utah, USA.
authors.forename: John F
authors.initials: JF
authors.lastname: Pohl
authors.affiliation: University of Minnesota Masonic Children's Hospital, Minneapolis, Minnesota, USA.
authors.forename: Sarah J
authors.initials: SJ
authors.lastname: Schwarzenberg
authors.affiliation: University of Texas Southwestern Medical School, Dallas, Texas, USA.
authors.forename: David
authors.initials: D
authors.lastname: Troendle
authors.affiliation: Medical College of Wisconsin, Milwaukee, Wisconsin, USA.
authors.forename: Steven
authors.initials: S
authors.lastname: Werlin
authors.affiliation: University of Iowa Carver College of Medicine, Iowa City, Iowa, USA.
authors.forename: Bridget
authors.initials: B
authors.lastname: Zimmerman
authors.affiliation: University of Iowa Carver College of Medicine, Iowa City, Iowa, USA.
authors.forename: Aliye
authors.initials: A
authors.lastname: Uc
authors.affiliation: Hospital for Sick Children, Toronto, Ontario, Canada.
authors.forename: Tanja
authors.initials: T
authors.lastname: Gonska
mesh_terms: D015746:Abdominal Pain / Q000175:diagnosis / Q000209:etiology; D000293:Adolescent; D001327:Autoimmune Diseases / Q000097:blood / Q000150:complications / Q000175:diagnosis / Q000628:therapy; D002648:Child; D002675:Child, Preschool; D003937:Diagnosis, Differential; D019468:Disease Management; D005938:Glucocorticoids / Q000627:therapeutic use*; D006801:Humans; D007074:Immunoglobulin G / Q000097:blood; D007391:International Cooperation; D041781:Jaundice, Obstructive / Q000175:diagnosis / Q000209:etiology; D008297:Male; D010179:Pancreas / Q000000981:diagnostic imaging / Q000276:immunology; D010186:Pancreatic Function Tests / Q000379:methods; D050500:Pancreatitis, Chronic / Q000150:complications / Q000175:diagnosis / Q000276:immunology / Q000628:therapy; D012042:Registries / Q000706:statistics & numerical data
chemical_list: D005938:Glucocorticoids; D007074:Immunoglobulin G