Autoimmune pancreatitis in children: working guidelines for diagnosis and treatment
(2016) The 5th world Congress of Pediatric Gastroenterology, Hepatology and Nutrition (WCPGHAN) — Location: Montréal, Canada (5.October.2016)
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- Background and aims Autoimmune pancreatitis (AIP) is an increasingly recognized and potential treatable cause of pancreatitis, but reports in children are limited to few cases. Until now, pediatric gastroenterologists relied on the adult AIP guidelines. However, first observations suggest that the presentation of AIP might be different in the children. Thus we aim to develop a working definition and diagnostic approach for AIP in children. Material and Methods Data about clinical symptoms, imaging, histology, other organ involvement findings and treatment modalities were collected using 2 different approaches: (1) a systematic literature search identifying pediatric cases of AIP and (2) children with an established diagnosis of AIP from the largest multicenter study of chronic pancreatitis in children (INSPPIRE) and Cliniques St-Luc (CUSL). Lastly we sought expert opinion from pediatric pancreatologists about AIP definition, diagnostic criteria and treatment. Results We identified 44 AIP cases, 26 from literature review, 14 from the INSPPIRE cohort study and 4 from CUSL. The median age of diagnosis of AIP was 13.2 years of age (range 2-17y). Abdominal pain (39/44, 87%) and/or obstructive jaundice (20/44, 45%) were the most frequently reported symptoms at diagnosis. Serum IgG4 levels, a hallmark in adult AIP, was above the upper limit of normal in only 8/38 (21%). Imaging findings were 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%). Of those for whom pancreas biopsy was performed, a combination of lymphoplasmacytic inflammation, pancreas fibrosis and ductal granulocyte infiltration was the main finding (18/25, 72%). Children with AIP had a prompt clinical response to steroids. Twenty-two percent of AIP patients developed other autoimmune/inflammatory conditions, mainly ulcerative colitis. Complications of AIP included impaired exocrine function requiring pancreatic enzyme replacement therapy (4/25,16%) and diabetes (3/27,11%). Conclusion AIP in children is a distinct subtype of pancreatitis. Children with AIP have (1) a high frequency of abdominal pain at diagnosis, (2) a lower frequency of positive serum IgG4, (3) parenchymal and/or duct abnormalities on cross-sectional imaging and (4) a combination of parenchymal lymphoplasmatic and granulocyte infiltration as well as fibrosis. A feature of the disease is the prompt clinical response to steroids. Based on these observations, we have established working guidelines to help identification and treatment of these children as well as to pave the way for prospective studies in the future.
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Scheers, I., Palermo, J., Freedman, S. J., Wilschanski, M., Abu-El-Haija, M., Fishman, D., Giefer, M., Heyman, M., Husain, S., Lin, T., Low, M., Morinville, V., Ooi, K., Perito, E., Schwarzenberg, S. J., Troendle, D., Uc, A., & Gonska, T. (2016). Autoimmune pancreatitis in children: working guidelines for diagnosis and treatment. The 5th world Congress of Pediatric Gastroenterology, Hepatology and Nutrition (WCPGHAN), Montréal, Canada.