Extensive chorioretinal atrophy in Vogt-Koyanagi-Harada disease

Purpose: To report extensive chorioretinal atrophy during the long-term cou rse of Vogt-Koyanagi-Harada (VKH) disease not treated properly in the initi al phase. Cases: Four patients with VKH disease were examined more than 10 years afte r onset of the disease. Observations: They presented initially with classic features of VKH disease , except 1 patient who had developed bilateral, acute angle-closure glaucom a as the initial sign. Two patients received systemic corticosteroid therap y at the acute phase of the disease. During the followup of 13-34 years sub sequent to onset, these patients had chronic recurrent anterior uveitis wit h apparently stable depigmented fundus. Eventually, they developed diffuse, extensive chorioretinal atrophy that resulted in severe visual loss. One p atient had an unusual familial occurrence of the disease. Conclusions: Failure to prescribe proper corticosteroid therapy in the init ial phase of VKH disease may lead to chronic recurrent uveitis. Long-standi ng uveitic reactions may eventually result in severe visual loss due to ext ensive chorioretinal degeneration. (C) 1999 Japanese Ophthalmological Socie ty.