Evidence and Consensus-Based Guidelines in Vogt-Koyanagi-Harada Disease

PURPOSE: To develop imaging and consensus-based guidelines for the application of multimodal imaging in the diagnosis, assessment of disease activity, and detection of complications in Vogt-Koyanagi-Harada (VKH) disease. DESIGN: Consensus agreement guided by systematic literature review and expert committee deliberation using a nominal group technique (NGT). PARTICIPANTS: International uveitis and retina specialists participating in the Multimodal Imaging in Uveitis (MUV) taskforce. METHODS: A panel of experts independently reviewed published literature and representative cases of active, resolved, and late-stage VKH disease, using multimodal imaging modalities: color fundus photography (CFP), OCT, fundus autofluorescence (FAF), fundus fluorescein angiography (FFA), indocyanine green angiography (ICGA), and OCT angiography (OCTA). Structured NGT sessions were conducted to define consensus-based imaging descriptors of active and resolved VKH disease, plus VKH complications including retinal pigment epithelium disruption, choroidal neovascularization (CNV), subretinal fibrosis, and other sequelae. MAIN OUTCOME MEASURES: Identification of reproducible multimodal imaging features of VKH and imaging criteria for determining disease activity and complications. RESULTS: The experts agreed that active VKH diagnosis is supported by characteristic imaging findings in the early stages, including bilateral multifocal serous retinal detachments and optic disc hyperemia on CFP, multiloculated subretinal fluid and choroidal thickening on OCT, and round-to-oval hypofluorescent dots on ICGA. Multimodal imaging was considered a critical adjunct to the clinical, neurologic, and integumentary findings incorporated in existing diagnostic systems for VKH. Beyond diagnosis, these modalities are essential for assessing disease activity. Both OCT and ICGA were considered sensitive in detecting disease activity, and subclinical/occult disease reactivation. Fundus fluorescein angiography is useful for detecting multiple pinpoint hyperfluorescent lesions and optic nerve head staining. In contrast, FAF was considered of limited utility for diagnosis. OCT and OCTA were also regarded as important tools for detecting complications such as subretinal fibrosis and CNV. CONCLUSIONS: Incorporating multimodal imaging findings, particularly CFP, OCT, and ICGA, into the evaluation and classification of VKH enhances diagnostic accuracy, improves assessment of disease activity, and enables earlier detection of vision-threatening complications. These consensus-based recommendations provide a structured framework for optimal multimodal imaging use in VKH and may inform future refinements of diagnostic criteria. FINANCIAL DISCLOSURE(S): Proprietary or commercial disclosure may be found in the Footnotes and Disclosures at the end of this article.