Authors
I. SCHEERS (1), J. PALERMO (2), S. FREEDMAN (3), M. WILSCHANSKI (4), U. SHAH (5), M. ABU-EL-HAIJA (2), B. BARTH (6), D. FISHMAN (7), C. GARIEPY (8), M. GIEFER (9), M. HEYMAN (10), R. HIMES (7), S. HUSAIN (11), T. LIN (2), Q. LIU (12), M. LOWE (11), M. MASCARENHAS (13), V. MORINVILLE (14), C. OOI (15), E. PERITO (10), D. PICCOLI (13), J. POHL (16), S. SCHWARZENBERG (17), D. TROENDLE (6), S. WERLIN (18), B. ZIMMERMAN (19), A. UC (19), T. GONSKA (20) / [1] Cliniques Universitaires Saint-Luc, , Belgium, Pediatric gastroenterology, hepatology and nutrition, [2] Cincinnati Children's Hospital Medical Center, Cincinnati/United States of America, , United States (the), , [3] Harvard Medical School, Beth Israel Deaconess Medical Center, Boston/United States of America, Boston, United States (the), , [4] Hadassah Hebrew University Hospital, Jerusalem/Israel, Jerusalem, Israel, , [5] Harvard Medical School, Massachusetts General Hospital for Children, Boston/United States of America, Boston, United States (the), , [6] University of Texas Southwestern Medical School, Dallas/United States of America, Dallas, United States (the), , [7] Baylor College of Medicine, Houston/United States of America, Houston, United States (the), , [8] Nationwide Children’s hospital, Columbus/United States of America, Columbus, United States (the), , [9] Seattle Children's Hospital, Seattle/United States of America, Seattle, United States (the), , [10] University of California at San Francisco, San Francisco/United States of America, San Francisco, United States (the), , [11] Children's Hospital of Pittsburgh of UPMC, Pittsburgh/United States of America, Pittsburg, United States (the), , [12] Keck School of Medicine, University of Southern California, Children's Hospital Los Angeles, Los Angeles/United States of America, Los Angeles, United States (the), , [13] The Children's Hospital of Philadelphia, Philadelphia/United States of America, Philadelphia, United States (the), , [14] Montreal Children’s Hospital, McGill University, Montreal/Canada, Montreal, Canada, , [15] Discipline of Paediatrics, School of Women’s and Children’s Health, Medicine, University of New South Wales and Sydney Children’s Hospital Randwick, Sydney/Australia, Sydney, Australia, , [16] University of Utah, Salt Lake City/United States of America, Salt lake City, United States (the), , [17] University of Minnesota Masonic Children’s Hospital, Minneapolis/United States of America, Minneapolis, United States (the), , [18] Medical College of Wisconsin, Milwaukee/United States of America, Milwaukee, United States (the), , [19] University of Iowa Carver College of Medicine, Iowa City/United States of America, Iowa City, United States (the), , [20] Hospital for Sick Children, Toronto/Canada, Toronto, United States (the),
Introduction
Autoimmune pancreatitis (AIP) is an increasingly recognized disease entity, but data in children are limited. Pediatric gastroenterologists relied on adult AIP guidelines but disease presentation and outcome of AIP in children might differ from the adult experience.
Aim
We aim to develop a working definition and diagnostic approach for AIP in children.
Methods
Clinical data, imaging, histology, and treatment modalities were collected using 2 different approaches: (1) a systematic literature search and (2) children with an AIP diagnosis from the largest multicenter study of chronic pancreatitis in children (INSPPIRE) and from Cliniques St-Luc (CUSL). We then sought expert opinion from pediatric pancreatologists.
Results
We identified 44 AIP cases, 26 from literature review, 14 from the INSPPIRE and 4 from CUSL cohort. The median age at diagnosis was 13.2 years. Abdominal pain (39/44, 87%) and/or obstructive jaundice (20/44, 45%) were the most reported symptoms at diagnosis. Elevated IgG4 levels was seen in only 8/38 (21%). Cross-sectional imaging was abnormal in all children mainly showing hypointense global or focal gland enlargement (35/43, 81%), irregularity of the main pancreatic duct (29/43, 67%) and common bile duct stricture (25/43, 58%). Lymphoplasmacytic inflammation, pancreas fibrosis and ductal granulocyte infiltration were the main histologic findings (18/25, 72%). Children with AIP had a prompt clinical response to steroids. Complications of AIP included impaired exocrine (4/25,16%) and/or endocrine (3/27,11%) function.
Conclusions
AIP in children is a distinct subtype of pancreatitis. Based on these observations, we established working guidelines to help identification and management of children with AIP and pave the way for future studies.
