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An Pediatr (Barc). 2013 Dec;79(6):381-4. doi: 10.1016/j.anpedi.2013.03.009. Epub 2013 Apr 19.

[Wegener's granulomatosis with anti-neutrophil cytoplasmic antibodies against anti-cathepsin G antigen].

[Article in Spanish]

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  • 1Servicio de Análisis Clínicos, Inmunología, Complejo Hospitalario de Jaén, Jaén, España. Electronic address:


Wegener's granulomatosis belongs to the group of small vessel vasculitis associated with anti-neutrophil cytoplasmic antibodies characterized by granulomatous inflammation and necrotising vasculitis in various organs with particular involvement of the upper and lower respiratory tracts and kidneys. Wegener's granulomatosis is a rare disorder in childhood and early diagnosis of this disease is critical to the long-term prognosis of the disease. The presence of positive cytoplasmic antineutrophil cytoplasmic antibody staining or a high titre of proteinase 3 antibodies were added as new criteria of vasculitis in childhood. This article presents a case of Wegener's granulomatosis, with the presence of anti-neutrophil cytoplasm antibodies with cytoplasmic pattern with absence of anti-proteinase 3 antibodies and presence of high levels of anti-cathepsin G antibodies, rarely described in Wegener's granulomatosis.

Copyright © 2012 Asociación Española de Pediatría. Published by Elsevier Espana. All rights reserved.


Anti-neutrophil cytoplasmic antibodies; Anticuerpos anti-catepsina G; Anticuerpos anticitoplasma de neutrófilo; Cathepsin-G antibodies; Granulomatosis de Wegener; Wegener's granulomatosis

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