Birdshot chorioretinopathy: Clinical characteristics and evolution

Abstract

During the period 1980-6 102 patients from 14 European eye clinics were diagnosed as having birdshot chorioretinopathy (BSCR). All were Caucasian, and the series consisted of 47 men and 55 women, with a mean age of 52·5 years. The major findings in this rare disorder concern the ocular fundus. Most marked are the patterned distribution of depigmented spots without hyperpigmentation, radiation from the optic disc in association with vitritis, retinal vasculopathy with frequent cystoid macular oedema, and involvement of the optic nerve head. The distribution and appearance of the lesions suggest that they are related to the major choroidal veins. Complications of the disease were epiretinal membranes, retinal neovascularisation, recurrent vitreous haemorrhage, subretinal neovascular membranes occurring both in the juxtapapillary and macular regions, and optic atrophy. The medical history was not contributary. HLA testing showed very strong disease association with HLA A29 (95·8%). The evidence suggests that it is a single disease entity rather than a group of disorders because of the remarkable similarity in the ophthalmological appearance and the clinical course, combined with the exceptionally high association with HLA A29.