Isabelle Scheers1,2, Joseph J Palermo3, Steven Freedman4, Michael Wilschanski5, Uzma Shah6, Maisam Abu-El-Haija3, Bradley Barth7, Douglas S Fishman8, Cheryl Gariepy9, Matthew J Giefer10, Melvin B Heyman11, Ryan W Himes8, Sohail Z Husain12, Tom K Lin3, Quin Liu13, Mark Lowe12, Maria Mascarenhas14, Veronique Morinville15, Chee Y Ooi16, Emily R Perito11, David A Piccoli14, John F Pohl17, Sarah J Schwarzenberg18, David Troendle7, Steven Werlin19, Bridget Zimmerman20, Aliye Uc20, Tanja Gonska1. 1. Hospital for Sick Children, Toronto, Ontario, Canada. 2. Cliniques Universitaires St-Luc, Brussels, Belgium. 3. Cincinnati Children's Hospital Medical Center, Cincinnati, Ohio, USA. 4. Harvard Medical School, Beth Israel Deaconess Medical Center, Boston, Massachusetts, USA. 5. Hadassah Hebrew University Hospital, Jerusalem, Israel. 6. Harvard Medical School, Massachusetts General Hospital for Children, Boston, Massachusetts, USA. 7. University of Texas Southwestern Medical School, Dallas, Texas, USA. 8. Baylor College of Medicine, Houston, Texas, USA. 9. Nationwide Children's Hospital, Columbus, Ohio, USA. 10. Seattle Children's Hospital, Seattle, Washington, USA. 11. University of California at San Francisco, San Francisco, California, USA. 12. Children's Hospital of Pittsburgh of UPMC, Pittsburgh, Pennsylvania, USA. 13. Keck School of Medicine, University of Southern California, Children's Hospital Los Angeles, Los Angeles, California, USA. 14. The Children's Hospital of Philadelphia, Philadelphia, Pennsylvania, USA. 15. Montreal Children's Hospital, McGill University, Montreal, Quebec, Canada. 16. 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. 17. University of Utah, Salt Lake City, Utah, USA. 18. University of Minnesota Masonic Children's Hospital, Minneapolis, Minnesota, USA. 19. Medical College of Wisconsin, Milwaukee, Wisconsin, USA. 20. University of Iowa Carver College of Medicine, Iowa City, Iowa, USA.
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.
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.
Authors: Phil A Hart; Terumi Kamisawa; William R Brugge; Jae Bock Chung; Emma L Culver; László Czakó; Luca Frulloni; Vay Liang W Go; Thomas M Gress; Myung-Hwan Kim; Shigeyuki Kawa; Kyu Taek Lee; Markus M Lerch; Wei-Chih Liao; Matthias Löhr; Kazuichi Okazaki; Ji Kon Ryu; Nicolas Schleinitz; Kyoko Shimizu; Tooru Shimosegawa; Roy Soetikno; George Webster; Dhiraj Yadav; Yoh Zen; Suresh T Chari Journal: Gut Date: 2012-12-11 Impact factor: 23.059
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Authors: Maisam Abu-El-Haija; Soma Kumar; Jose Antonio Quiros; Keshawadhana Balakrishnan; Bradley Barth; Samuel Bitton; John F Eisses; Elsie Jazmin Foglio; Victor Fox; Denease Francis; Alvin Jay Freeman; Tanja Gonska; Amit S Grover; Sohail Z Husain; Rakesh Kumar; Sameer Lapsia; Tom Lin; Quin Y Liu; Asim Maqbool; Zachary M Sellers; Flora Szabo; Aliye Uc; Steven L Werlin; Veronique D Morinville Journal: J Pediatr Gastroenterol Nutr Date: 2018-01 Impact factor: 2.839
Authors: Aliye Uc; Emily R Perito; John F Pohl; Uzma Shah; Maisam Abu-El-Haija; Bradley Barth; Melena D Bellin; Kate M Ellery; Douglas S Fishman; Cheryl E Gariepy; Matthew J Giefer; Tanja Gonska; Melvin B Heyman; Ryan W Himes; Sohail Z Husain; Asim Maqbool; Maria R Mascarenhas; Brian A McFerron; Veronique D Morinville; Tom K Lin; Quin Y Liu; Jaimie D Nathan; Sue J Rhee; Chee Y Ooi; Zachary M Sellers; Sarah Jane Schwarzenberg; Jose Serrano; David M Troendle; Steven L Werlin; Michael Wilschanski; Yuhua Zheng; Ying Yuan; Mark E Lowe Journal: Pancreas Date: 2018 Nov/Dec Impact factor: 3.327
Authors: 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 Journal: J Pediatr Gastroenterol Nutr Date: 2018-08 Impact factor: 2.839