Wegener's granulomatosis: The ocular manifestations revisited

AIM . To investigate whether the extent of ocular morbidity had de-creased as a result of improved diagnosis, awareness and treatment of Wegener 's granulomatosis (WG). MATERIALS AND METHODS . A retrospective study of all patients with ophthalmic involvement due to WG over an 8-year period. RES...

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Bibliographic Details
Published in:Orbit (Amsterdam) 2000, Vol.19 (4), p.253-261
Main Authors: Sadiq, S.A., Jennings, C.R., Jones, N.S., Downes, R.N.
Format: Article
Language:English
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Summary:AIM . To investigate whether the extent of ocular morbidity had de-creased as a result of improved diagnosis, awareness and treatment of Wegener 's granulomatosis (WG). MATERIALS AND METHODS . A retrospective study of all patients with ophthalmic involvement due to WG over an 8-year period. RESULTS . Forty-nine patients were diagnosed to have WG. Of these, 28 had ocular involvement: 21 patients had focal ocular involvement (conjunctivitis, episcleritis, scleritis, keratitis, iritis, retinitis)and 7 had orbital involvement. Permanent visual loss occurred in three patients with orbital involvement, but in no patients with focal ocular disease. Up to 90% of patients had systemic involvement. Three deaths oc- curred among those with ocular involvement. CONCLUSIONS . Patients with WG have a much improved visual prog- nosis as a result of early diagnosis and intervention with systemic im- munosuppression. A combination of assays for ANCA and tissue biop-sies were needed to establish the diagnosis of WG. Treatment with immunosuppressive agents (usually prednisolone and cyclophospha- mide)led to a good response in the majority of cases.
ISSN:0167-6830
1744-5108
DOI:10.1076/orbi.19.4.253.2654