Abstract
PURPOSE: It is crucial to subdivide Vogt-Koyanagi-Harada (VKH) disease into two subentities, initial-onset disease versus chronically evolving disease. For early diagnosis and precise follow-up of VKH choroiditis, the "Revised criteria for VKH" are no more sufficient for the appraisal of VKH and new biomarkers for disease activity are needed. It has been shown that, if initial-onset disease is treated promptly within the "therapeutic window of opportunity" and long enough with dual steroidal and non-steroidal immunosuppression, the disease can be cured in a large proportion of cases, an approach still contested. The proportion of chronic evolution and/or sunset-glow fundus (SGF) following steroidal monotherapy versus dual steroidal and non-steroidal immunosuppression was compared. METHODS: A literature search was performed, identifying studies on initial-onset VKH treated either by steroidal monotherapy or dual immunosuppression. Evolution toward chronicity and/or SGF was compared in both groups. RESULTS: Twenty studies were identified with reported long-term outcomes. In 16 studies, 802 patients received steroidal monotherapy, while in 4 studies, 172 patients received dual steroidal and non-steroidal immunosuppression. Chronic evolution and SGF occurred, respectively, in 44% and 59% in the corticosteroid-alone group versus 2.3% and 17.5% in the dual therapy group with no chronic evolution in three studies and no SGF in two studies. CONCLUSIONS: Chronic evolution and SGF are significantly less frequent in initial-onset VKH when treated with immediate dual steroidal and non-steroidal immunosuppression with a high proportion of healed cases. This combined approach seems recommended in the management of initial-onset VKH disease.