Autoimmune Pancreatitis in Children: Characteristic Features, Diagnosis, and Management

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.

EPC/HPSG evidence-based guidelines for the management of pediatric pancreatitis

“…4. The optimal intervention strategy in cases of suspected or confirmed infected necrotizing pancreatitis is initially imaging-guided percutaneous (retroperitoneal) catheter drainage or endoscopic transluminal drainage, which, if necessary, can be followed by endoscopic or surgical necrosectomy.…”

“…Having a significant effect on the life of the children and parents PP requires up-to-date and evidence -based was founded in 2010 described the first ever pediatric definition of AP, ARP and CP 3 and developed consensus guidelines for evaluation of pediatric ARP, CP and its risk factors [4][5][6] . Hungarian Pancreatic Study Group (HPSG) developed the first evidence-based medicine (EBM) management guideline in PP in 2014 and published it in 2015 in Hungarian 7 .…”

Autoimmune Pancreatitis in an Adolescent: A Diagnostic Challenge

“…Type 2 occurs in younger adults with normal IgG4 levels and no systemic manifestations, except for inflammatory bowel disease. Pediatric AIP is a unique form of the disease with some similarity to type 2 AIP in adults [4,5].…”

“…The diagnosis of pediatric AIP, according to the recent guidelines, relies on the combination of clinical symptoms (usually abdominal pain, jaundice, weight loss, fatigue, and vomiting in contrast to painless jaundice in adults) and imaging findings, sometimes complemented with histopathology [4].…”

“…The first pediatric case was reported in 2008. However, to date, there are few pediatric case series described in the literature, and international recommendations for the approach to AIP have been released recently [3][4][5][6].…”