Introduction
Autoimmune pancreatitis represents 2 % of chronic pancreatitis , most often in adult male presentation associated with IgG4. We present a case of a patient 6 years old, with a dependent pancreatic and bile duct obstruction abdominal mass, which is interesting because this presentation in children is more common with autoimmune pancreatitis that malignancies.
Objectives
Show an exceptional case in pediatrics rheumatology.
Methods
Presentation of case.
Results
Female 6 years old, previously healthy, 3-year evolution gastric recurrent vomiting, abdominal pain intermittently mesogastrio , adding hiporexia, jaundice and increased waist circumference, with palpable mass in the right upper quadrant. Cholestatic syndrome, 857 Lipase, amylase 137 Immunoglobulin IgG subclass 4: 23mg/dL (5-6 years: 1-121). CT: dilatation of intra and extra hepatic bile duct. Level space-occupying hepatic hilum extending to pancreatic head and esophagogastric junction mass. Gonadotropin and alpha-fetoprotein: negatives. Cholecystectomy, incisional biopsy of tumor, liver biopsy was performed. He reported: lymphoplasmacytic sclerosing pancreatitis (chronic autoimmune pancreatitis).
Conclusion
This is a rare autoimmune disorder that resembles a pancreatic neoplasm with biliary obstruction, occurring primarily in adults, making it an exceptional case in pediatrics. As part of an IgG4 -associated systemic disease, serum level may be normal in up to 40 %, being positive in pancreatic tissue.
Disclosure of interest
None declared.