Pediatric Autoimmune Pancreatitis: A Case of Obstructive Jaundice

Introduction Autoimmune pancreatitis (AIP) is a rare, distinct form of chronic pancreatitis [1], categorized into Type 1, often associated with elevated IgG4, and Type 2, which is less related to IgG4 and involves more localized ductal changes. In pediatric cases, AIP often lacks elevated IgG4, making diagnosis dependent on imaging findings and response to steroid therapy [2]. Case Presentation An 11-year-old boy presented with a one-month history of weight loss and fatigue, along with a few days of jaundice, pruritus, epigastric pain and dark urine. Laboratory results showed significantly elevated liver and pancreatic enzymes, with normal IgG and IgG4 levels. Imaging demonstrated biliary dilation, pancreatic head enlargement and main pancreatic duct compression. Ultrasound and MRI findings were consistent with AIP, with a suspected diagnosis of pancreas divisum (PD) and annular pancreas (AP). AP was further suspected during Endoscopic Retrograde Cholangiopancreatography (ERCP). Type 2 AIP was diagnosed based on clinical features, elevated enzymes, imaging and a positive response to corticosteroid therapy [3]. Treatment with prednisolone (1 mg/kg/day) and pancreatic enzyme supplementation led to symptom resolution and improved laboratory markers. Discussion Type 2 AIP is rare in children but should be considered in obstructive jaundice cases. In pediatric AIP, elevated IgG4 levels are often absent, diagnosis relies on imaging findings, elevated pancreatic and liver enzymes and positive response to corticosteroids [3]. Anatomical anomalies like PD (4% prevalence) and AP (0.01% prevalence) can impair drainage and contribute to inflammation [4,5]. These anomalies may coexist with AIP, mimic its features or be mistakenly diagnosed as AIP has similar symptoms. Since steroid therapy leads to rapid improvement, AIP is likely the primary condition, with anatomical anomalies either coexisting or being misdiagnosed initially. Conclusions This case highlights the significance of diagnosing AIP in pediatric patients presenting with obstructive jaundice. Early detection followed by swift steroid treatment resulted in notable clinical improvement, emphasizing the necessity of prompt intervention. Further research is needed to better understand the pathophysiology and longterm outcomes of pediatric AIP.