Extensive Chorioretinal Atrophy in Vogt–Koyanagi–Harada Disease

Purpose: To report extensive chorioretinal atrophy during the long-term course of Vogt–Koyanagi–Harada (VKH) disease not treated properly in the initial phase. Cases: Four patients with VKH disease were examined more than 10 years after onset of the disease. Observations: They presented initially with classic features of VKH disease, except 1 patient who had developed bilateral, acute angle-closure glaucoma as the initial sign. Two patients received systemic corticosteroid therapy at the acute phase of the disease. During the follow-up of 13–34 years subsequent to onset, these patients had chronic recurrent anterior uveitis with apparently stable depigmented fundus. Eventually, they developed diffuse, extensive chorioretinal atrophy that resulted in severe visual loss. One patient had an unusual familial occurrence of the disease. Conclusions: Failure to prescribe proper corticosteroid therapy in the initial phase of VKH disease may lead to chronic recurrent uveitis. Long-standing uveitic reactions may eventually result in severe visual loss due to extensive chorioretinal degeneration.