Treatment and prognosis of Vogt–Koyanagi–Harada disease: real-life experience in long-term follow-up

Abstract: Background: Vogt–Koyanagi–Harada (VKH) disease is a form of uveitis that is rare in Western countries. The aim of this study was to report on the long-term real-life treatment and prognosis of VKH in Italy. Methods: The clinical features, complications, and final visual acuity were retrospectively evaluated in 38 patients with VKH (mean follow-up: 120 months) globally, according to oral or intravenous corticosteroid treatment at onset and subsequent immunosuppressive therapy. Results: The mean final visual acuity was 0.13 ± 0.4 logMAR, which was a significant increase from the baseline (p < 0.0001). The patients who received intravenous rather than oral corticosteroids relapsed less (p = 0.026), with fewer relapses/patient/month of follow-up (p < 0.0001), and showed less frequent sunset glow fundus (33.3% versus 55%) and more relapse-free cases after induction therapy (p = 0.007). Delayed immunosuppressive therapy (median: 180 days from the onset of symptoms) reduced the rate of sunset glow fundus. The onset of sunset glow fundus was associated with a worse final visual acuity (p = 0.006). Conclusion: The long-term prognosis of VKH is quite good. Intravenous corticosteroids given at the onset of VKH are more effective than oral corticosteroids. Even if it is not given immediately after symptoms onset, immunosuppressive therapy is able to reduce the incidence of sunset glow fundus and to improve the final visual prognosis.