Volume 12 Supplement 1

Proceedings of the 21st European Pediatric Rheumatology (PReS) Congress

Open Access

Case autoimmune pancreatitis in children

  • Alicia G Cazares Camacho1,
  • Maria Maldonado1,
  • Enrique Faugier1 and
  • Maria de Lourdes Cabrera2
Pediatric Rheumatology201412(Suppl 1):P287

https://doi.org/10.1186/1546-0096-12-S1-P287

Published: 17 September 2014

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.

Authors’ Affiliations

(1)
Reumatologia pediatrica, Hospital Infantil de Mexico Federico Gomez
(2)
Patologia, Hospital Infantil de Mexico Federico Gomez

Copyright

© Cazares Camacho et al; licensee BioMed Central Ltd. 2014

This article is published under license to BioMed Central Ltd. This is an Open Access article distributed under the terms of the Creative Commons Attribution License (http://creativecommons.org/licenses/by/4.0), which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited. The Creative Commons Public Domain Dedication waiver (http://creativecommons.org/publicdomain/zero/1.0/) applies to the data made available in this article, unless otherwise stated.

Advertisement