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

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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 (NGT) 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 (SUN) diagnostic criteria. Imaging modalities included color fundus photography (CFP), fundus fluorescein angiography (FFA), optical coherence tomography (OCT), fundus autofluorescence (FAF), indocyanine green angiography (ICGA), and OCT angiography (OCTA). NGT 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. FFA 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. FFA and OCT assist in demonstrating disease resolution, and detection of late changes such as retinal non-perfusion, 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. CONCLUSION: Incorporation of consensus-based imaging guidelines by MUV, particularly CFP, FFA and OCT, enhances diagnostic evaluation, 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.

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