Ocular presentation in Wegener granulomatosis
Torres, R.M.; Herreras, J.M.; Becerra, E.; Blanco, G.; Méndez, M.C.; Saornil, M.A.
Archivos de la Sociedad Espanola de Oftalmologia 79(3): 135-138
2004
ISSN/ISBN: 0365-6691 PMID: 15045656 Document Number: 569172
A 71 year-old female presented with bilateral conjunctivitis, scleritis and peripheral ulcerative keratitis. Ancillary tests showed presence of anti-neutrophil cytoplasmic antibodies, with diffuse granular cytoplasmic immunofluorescence staining (ANCAc). Conjunctival biopsy showed necrotizing vasculitis in small vessels. Diagnosis of Wegener Granulomatosis (WG) was established and upper respiratory affection detected. Treatment with megadoses of cyclophosphamide and corticoids was initiated and remission of the disease was obtained. WG is a potentially lethal systemic vasculitis in which ocular involvement may be the first clinical presentation. Scleritis and peripheral ulcerative keratitis indicate systemic involvement, highlighting the importance of prompt diagnosis and treatment.