Subretinal fibrosis in patients with Vogt-Koyanagi-Harada disease

Objective: To describe subretinal fibrosis as a long-term complication of Vogt-Koyanagi-Harada (VKH) disease. Design: Retrospective, clinic-based, cross-sectional study and clinical correlation. Participants: Ten patients with VKH disease and subretinal fibrosis were seen at two uveitis referral centers between 1977 and 1997. Intervention: A review of the historical, clinical, and fluorescein angiographic features was performed. Main Outcome Measures: The prevalence, demographic and clinical features, and time to development of subretinal fibrosis were summarized. Results: Subretinal fibrosis occurred in 20 eyes of 10 patients with VKH disease, an overall prevalence of 8% between the two institutions. Patient age ranged from 16 years to 48 years, with a median of 34.5 years. Five patients were Hispanic, one was mixed Hispanic and American Indian, three were Asian or mixed Asian and Caucasian, and one was African-American. Eight of the 10 patients were men. All patients were in the chronic, recurrent phase of their disease when they had subretinal fibrosis develop, and all patients had recurrent episodes of posterior uveitis. Presenting vision ranged from 20/20 to light perception, with a median acuity of 20/200. All patients were initially treated with oral and topical corticosteroids. Four patients required additional noncorticosteroid immunosuppressive therapy. Time from diagnosis of VKH disease to development of subretinal fibrosis ranged from zero (fibrosis present at time of diagnosis) to 27 years, with a median time of 10 months. The median time from diagnosis of VKH to development of subretinal fibrosis in Hispanic patients was 6.5 months, whereas in non-Hispanic patients it was 6.5 years. Final vision ranged from 20/25 to light perception, with a median acuity of 20/60. Seven of 20 eyes had a final visual acuity of 20/40 or better, and seven eyes saw 20/200 or worse. Five of the eyes with 20/200 or worse vision had fibrosis involving the fovea. Conclusions: Subretinal fibrosis occurs in a sizeable proportion of patients with VKH disease and may contribute to permanent loss of vision.