Vogt-Koyanagi-Harada disease: Recent perspectives

Vogt-Koyanagi-Harada (VKH) disease is a dynamic immune-mediated stromal choroiditis induced by dysregulated T cells targeting melanocytes. This disease is a unification of varied ocular and systemic features documented over the centuries across different parts of the world. With striking ethnic and geographic variations, VKH remains one of the leading causes of noninfectious uveitis. Recent advances in the immunopathogenesis with noted key pathogenic pathways including activation of the IL-23/IL-17 axis, impaired regulatory T-cell function with reduced expression of IL-35 levels, elevated IL-6 levels, epigenetic regulation mediated by microRNAs, and emerging evidence in the gut microbiome dysbiosis pave the way for early, targeted immunomodulatory strategies. Parallel development in the multimodal imaging front has transformed the diagnostic paradigm as to early detection of choroidal inflammation, identification of subclinical activity, and objective monitoring of the disease. Treatment emphasizes early initiation of steroid-sparing immunosuppression to prevent chronic progression and late-stage changes. Targeted approaches like biologics and Janus kinase inhibitors offer promising outcomes in refractory cases. This review integrates epidemiological perspectives, evolving immunopathogenesis, newer classifications, atypical presentations, utilization of multimodal imaging, and the therapeutic advances in VKH.