SUMMARYThis study was undertaken to assess the efficacy of a stan dard regime of high-dose systemic oral corticosteroids in the management of retinal vasculitis. The study was per formed because the single most common reason for refer ral to our specialist clinic is the apparent failure of patients to respond to a course of systemic steroids, which in most cases appeared to be due to an inadequate initial dose. A retrospective study of 29 patients (30 treatment episodes) with sight-threatening retinal vasculitis man aged initially with high-dose systemic steroids was eval uated 1 year after treatment. Patients included in the study all started treatment with � 1 mg/kg prednisolone and remained on a high steroid dose (�40 mg predniso lone) for at least 5 weeks. No patient was on any other immunosuppressive agent at the start of the study. Thera peutic success for this regime, as judged by improvement in visual acuity, was 60%, improving to 77% with addi tion of other immunosuppressive agents. Eight patients required additional immunosuppressives. Although documented side-effects of steroids were common (50% of cases managed on steroids alone), in only 5 patients were they therapeutically important. Tw elve of the 22 patients managed on high-dose steroids alone were off treatment at 12 months. There was no correlation at any stage between visual acuity, activity index or relapses and the final visual outcome at 12 months. Seven cases had a poor visual outcome and the causes for this included relapse in the twelfth month of follow-up, persistent cys toid macular oedema and lens opacity. These results sug gest that high-dose oral steroids should be tried in the initial management of such patients before contemplating other more complicated regimes or accepting a poor visual outcome.
Birdshot chorioretinopathy is a rare inflammatory disorder with an insidious onset that can slowly progress to severe visual loss. The pathogenesis is unknown. This study used indocyanine green (ICG) angiography to investigate the degree of choroidal vascular involvement with progression of disease and to determine the nature of the birdshot lesions. Seven patients with birdshot chorioretinopathy had ICG angiography performed with a scanning laser ophthalmoscope at various stages of clinical disease. Results were compared with fluorescein fundal angiography (FFA). All large choroidal vessels appeared normal. The birdshot lesions were demonstrated with ICG but not with FFA and were represented by dark areas on ICG angiography. Typically these areas were bordered by large or medium-sized choroidal vessels and their appearance suggested small choroidal vessel hypoperfusion. In disease of recent onset, some lesions masked fluorescence from large underlying choroidal vessels possibly due to inflammatory choroidal infiltrates. In long-standing disease, the choroidal angioarchitecture was relatively normal within the birdshot lesions. This study of birdshot chorioretinopathy demonstrates abnormalities in the small choroidal vessels within the birdshot lesions. ICG angiography detects the birdshot lesions more readily than FFA and may be of benefit in assessing disease activity.
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