Table of Contents Author Guidelines Submit a Manuscript
Case Reports in Pediatrics
Volume 2017, Article ID 2962794, 5 pages
https://doi.org/10.1155/2017/2962794
Case Report

The Diagnosing Challenge of a Positive ANCA Vasculitis in the Paediatric Age

1Paediatric and Neonatal Intensive Care Service, Centro Materno Infantil do Norte, Centro Hospitalar do Porto, Porto, Portugal
2Paediatric Service, Hospital da Senhora da Oliveira, Guimarães, Portugal
3Otorhinolaryngology Service, Centro Hospitalar de Vila Nova de Gaia/Espinho, Vila Nova de Gaia, Portugal
4Paediatric Service, Centro Materno Infantil do Norte, Centro Hospitalar do Porto, Porto, Portugal
5Department of Paediatric Nephrology, Paediatric Service, Centro Materno Infantil do Norte, Centro Hospitalar do Porto, Porto, Portugal

Correspondence should be addressed to Clara Preto; moc.liamtoh@oterpmaralc

Received 29 July 2017; Revised 10 October 2017; Accepted 23 November 2017; Published 19 December 2017

Academic Editor: Alberto Spalice

Copyright © 2017 Clara Preto et al. This is an open access article distributed under the Creative Commons Attribution License, which permits unrestricted use, distribution, and reproduction in any medium, provided the original work is properly cited.

Abstract

ANCA-positive systemic vasculitides, rare in paediatric age, present multiorganic involvement. A female teenager presented with a history of subglottic stenosis diagnosed at the age of 12. From the investigation carried out, we highlight hematoproteinuria and negative ANCAs. At 15 years old, she was admitted for gastrointestinal symptoms and respiratory distress. She presented poor peripheral perfusion, pulmonary haemorrhage, respiratory failure, and severe renal insufficiency. She was started mechanical ventilation and emergency haemodialysis. The immunological study revealed ANCA MPO positive. A presumptive diagnosis of ANCA-positive vasculitis was made, and she was started corticotherapy, cyclophosphamide, and plasmapheresis. A renal biopsy, performed later, showed crescentic glomerulonephritis with chronicity signs. Positive ANCA vasculitis may progress slowly or suddenly. The diagnosis was confirmed by a biopsy; however, we can make a presumptive diagnosis based on clinical findings and in a positive ANCA test in order to start an early treatment and decrease the associated morbimortality.