TY - JOUR
T1 - Autoimmune Pancreatitis in Children
T2 - Characteristic Features, Diagnosis, and Management
AU - Scheers, Isabelle
AU - Palermo, Joseph J.
AU - Freedman, Steven
AU - Wilschanski, Michael
AU - Shah, Uzma
AU - Abu-El-Haija, Maisam
AU - Barth, Bradley
AU - Fishman, Douglas S.
AU - Gariepy, Cheryl
AU - Giefer, Matthew J.
AU - Heyman, Melvin B.
AU - Himes, Ryan W.
AU - Husain, Sohail Z.
AU - Lin, Tom K.
AU - Liu, Quin
AU - Lowe, Mark
AU - Mascarenhas, Maria
AU - Morinville, Veronique
AU - Ooi, Chee Y.
AU - Perito, Emily R.
AU - Piccoli, David A.
AU - Pohl, John F.
AU - Schwarzenberg, Sarah J.
AU - Troendle, David
AU - Werlin, Steven
AU - Zimmerman, Bridget
AU - Uc, Aliye
AU - Gonska, Tanja
N1 - Publisher Copyright:
© 2017 by the American College of Gastroenterology.
PY - 2017/10/1
Y1 - 2017/10/1
N2 - 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.
AB - 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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U2 - 10.1038/ajg.2017.85
DO - 10.1038/ajg.2017.85
M3 - Article
C2 - 28374818
AN - SCOPUS:85017109823
SN - 0002-9270
VL - 112
SP - 1604
EP - 1611
JO - American Journal of Gastroenterology
JF - American Journal of Gastroenterology
IS - 10
ER -