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 & 8 others David A. Piccoli, John F. Pohl, Sarah J. Schwarzenberg, David Troendle, Steven Werlin, Bridget Zimmerman, Aliye Uc, Tanja Gonska

Research output: Contribution to journalArticle

15 Citations (Scopus)

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.

Original languageEnglish (US)
Pages (from-to)1604-1611
Number of pages8
JournalAmerican Journal of Gastroenterology
Volume112
Issue number10
DOIs
StatePublished - Oct 1 2017

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Pancreatitis
Obstructive Jaundice
Pancreatic Ducts
Common Bile Duct
Disease Management
Granulocytes
Abdominal Pain
Multicenter Studies
Registries
Pancreas
Histology
Pathologic Constriction
Fibrosis
Cross-Sectional Studies
Immunoglobulin G
Steroids
Databases
Pediatrics
Inflammation
Serum

Cite this

Scheers, I., Palermo, J. J., Freedman, S., Wilschanski, M., Shah, U., Abu-El-Haija, M., ... Gonska, T. (2017). Autoimmune Pancreatitis in Children: Characteristic Features, Diagnosis, and Management. American Journal of Gastroenterology, 112(10), 1604-1611. https://doi.org/10.1038/ajg.2017.85

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

In: American Journal of Gastroenterology, Vol. 112, No. 10, 01.10.2017, p. 1604-1611.

Research output: Contribution to journalArticle

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 2017, 'Autoimmune Pancreatitis in Children: Characteristic Features, Diagnosis, and Management', American Journal of Gastroenterology, vol. 112, no. 10, pp. 1604-1611. https://doi.org/10.1038/ajg.2017.85
Scheers I, Palermo JJ, Freedman S, Wilschanski M, Shah U, Abu-El-Haija M et al. Autoimmune Pancreatitis in Children: Characteristic Features, Diagnosis, and Management. American Journal of Gastroenterology. 2017 Oct 1;112(10):1604-1611. https://doi.org/10.1038/ajg.2017.85
Scheers, Isabelle ; Palermo, Joseph J. ; Freedman, Steven ; Wilschanski, Michael ; Shah, Uzma ; Abu-El-Haija, Maisam ; Barth, Bradley ; Fishman, Douglas S. ; Gariepy, Cheryl ; Giefer, Matthew J. ; Heyman, Melvin B. ; Himes, Ryan W. ; Husain, Sohail Z. ; Lin, Tom K. ; Liu, Quin ; Lowe, Mark ; Mascarenhas, Maria ; Morinville, Veronique ; Ooi, Chee Y. ; Perito, Emily R. ; Piccoli, David A. ; Pohl, John F. ; Schwarzenberg, Sarah J. ; Troendle, David ; Werlin, Steven ; Zimmerman, Bridget ; Uc, Aliye ; Gonska, Tanja. / Autoimmune Pancreatitis in Children : Characteristic Features, Diagnosis, and Management. In: American Journal of Gastroenterology. 2017 ; Vol. 112, No. 10. pp. 1604-1611.
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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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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

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AU - Zimmerman, Bridget

AU - Uc, Aliye

AU - Gonska, Tanja

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