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.

Reactivation of previously controlled Vogt–Koyanagi–Harada disease more than 46 years following COVID-19 vaccination: a case study

“…22 Nakayama et al 3 reported that anterior and/or posterior segment recurrence of inflammation was observed in 25 (22.5%) of 111 patients. Accorinti et al 21 reported lower recurrence in patients who received intravenous than oral corticosteroids. Our patient's treatment was not maintained because it had been initiated 46 years previously.…”

“…Accorinti et al. 21 reported lower recurrence in patients who received intravenous than oral corticosteroids. Our patient’s treatment was not maintained because it had been initiated 46 years previously.…”

“…20 and Accorinti et al. 21 reported that 9 (23.1%) of 39 eyes and 17 (44.7%) of 38 patients treated with systemic corticosteroids, respectively, developed sunset glow fundus, which frequently occurs after treatment in patients with VKH disease. Additionally, Nakayama et al.…”

Long-term recurrence interval of Vogt–Koyanagi–Harada disease post-COVID-19 vaccination: A case study

“…While Hirooka et al [13] reported 9 of 39 eyes (23.1%) treated with systemic corticosteroids and Accorinti et al observed 17 of 38 (44.7%) patients treated with systemic corticosteroids [14], Nakayama et al [3] found sunset glow fundus, which frequently occurs after treatment in VKH patients, in 110 (49.5%) eyes treated with pulse intravenous corticosteroids. According to Hirooka et al, a signi cantly higher number of recurrences happen within 12 months in the sunset than in the non-sunset glow fundus [13]; our patient had a recurrence after 46 years.…”

Vogt‐Koyanagi‐Harada disease in pregnancy: Case report and review of 32 patients in the literature

“…The diagnosis is based on characteristic clinical manifestations, as described above, and the exclusion of other entities of uveitis: not only infectious uveitis caused by syphilis and tuberculosis but also non‐infectious uveitis in association with tubulointerstitial nephritis 9 and inflammatory bowel disease 10 and so on. The standard of care for Vogt‐Koyanagi‐Harada disease is steroid pulse therapy with methylprednisolone 1000 mg daily for 3 days, followed by tapering of oral prednisolone in half a year 11 . Vogt‐Koyanagi‐Harada disease may occur in pregnancy and pose a problem how to apply the standard of care in a pregnant woman 12–14 .…”