Evidence and Consensus Based Imaging Guidelines in Behçet Disease Uveitis. Multimodal Imaging in Uveitis (MUV) Taskforce Report 18

PURPOSE: To develop imaging and consensus-based guidelines for the application of multimodal imaging in the clinical diagnosis, monitoring, and detection of complications in Behçet disease (BD) uveitis. DESIGN: International expert consensus agreement using the nominal group technique guided by systematic literature review. PARTICIPANTS: International uveitis and retina experts participating in the Multimodal Imaging in Uveitis (MUV) taskforce. METHODS: A committee of experts reviewed the published literature on imaging in BD uveitis, along with representative multimodal imaging datasets of active, resolved and late-stage uveitis in BD. All cases in these datasets met the Standardized Uveitis Nomenclature diagnostic criteria. Imaging modalities included color fundus photography (CFP), fundus fluorescein angiography (FFA), OCT, fundus autofluorescence (FAF), indocyanine green angiography (ICGA), and OCT angiography (OCTA). Nominal group technique sessions were conducted to define consensus-based key imaging descriptors of active and resolved BD uveitis, along with the complications and sequelae. MAIN OUTCOME MEASURES: Identification of reproducible multimodal imaging features of active and resolved BD uveitis. RESULTS: The experts agreed that CFP, FFA, and OCT are the most relevant imaging modalities in the management of BD uveitis, with particular emphasis on ultra-widefield imaging. Characteristic findings on CFP include vitreous haze, retinal infiltrates, optic nerve head inflammation, and prominent retinal vasculitis with possible occlusion. Fundus fluorescein angiography was deemed critical in assessing vascular, macular, and optic nerve head leakage, thereby indicating disease activity. OCT is helpful in detecting and characterizing cystoid macular edema, partial thickness inner retinitis (smudge-sign), subretinal fluid, and overlying vitreous condensation. Fundus fluorescein angiography and OCT assist in demonstrating disease resolution, and detection of late changes such as retinal nonperfusion, neovascularization, epiretinal membrane formation, and retinal atrophy. The experts concluded that FAF, ICGA, and OCTA have limited role in disease. Based on these findings, consensus-based statements were generated and voted upon by the MUV taskforce. CONCLUSIONS: Incorporation of consensus-based imaging guidelines, particularly CFP, FFA, and OCT, enhances diagnostic evaluation and improves assessment of disease activity, assessment of treatment response, and detection of complications in BD uveitis. These recommendations provide a structured framework for optimal multimodal imaging use in BD uveitis and aid 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.