title: [Wegener's granulomatosis with anti-neutrophil cytoplasmic antibodies against anti-cathepsin G antigen].
abstract: 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.
authors.affiliation: Servicio de Análisis Clínicos, Inmunología, Complejo Hospitalario de Jaén, Jaén, España. Electronic address: eocanap@gmail.com.
authors.forename: E
authors.initials: E
authors.lastname: Ocaña Pérez
authors.forename: A M
authors.initials: AM
authors.lastname: Peña Casas
authors.forename: T
authors.initials: T
authors.lastname: del Campo Muñoz
authors.forename: A
authors.initials: A
authors.lastname: Avila Casas
authors.forename: R
authors.initials: R
authors.lastname: Luque Barona
mesh_terms: D019268:Antibodies, Antineutrophil Cytoplasmic / Q000097:blood*; D056649:Cathepsin G / Q000276:immunology*; D002648:Child; D014890:Granulomatosis with Polyangiitis / Q000097:blood* / Q000276:immunology*; D006801:Humans; D008297:Male
chemical_list: D019268:Antibodies, Antineutrophil Cytoplasmic; D056649:Cathepsin G
keywords: Anti-neutrophil cytoplasmic antibodies; Anticuerpos anti-catepsina G; Anticuerpos anticitoplasma de neutrófilo; Cathepsin-G antibodies; Granulomatosis de Wegener; Wegener's granulomatosis