Wegener's granulomatosis: The ocular manifestations revisited

Abstract

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.