Retinal vasculitis severity assessment: intra- and inter-observer reliability of a new scheme for grading wide-field fluorescein angiograms in retinal vasculitis

Behcet's syndrome is a multisystem inflammatory disorder characterized by a chronic relapsing course and diverse clinical manifestations, prominently affecting young adults along the ancient Silk Road and beyond. The disease pathogenesis involves complex interactions between genetic predisposition, environmental triggers, and dysregulated immune responses, leading to systemic vasculitis and tissue damage. Ocular involvement, a hallmark of Behcet's Disease (BD), significantly impacts morbidity, with uveitis as a common initial presentation that can progress to severe vision-threatening complications like retinal vasculitis and occlusive disease. This review consolidates current knowledge on ocular manifestations in BD, emphasizing the pivotal role of multimodal imaging in diagnostic evaluation and management. Fundus photography serves as a baseline tool for documenting intraocular lesions and monitoring treatment responses. Fluorescein angiography remains the gold standard for detecting acute inflammatory changes and vascular leakage patterns essential for disease staging and prognostication. Recent advancements in imaging, such as ultra-wide field imaging (UWF), indocyanine green angiography (ICGA), Doppler ultrasonography, Optical Coherence Tomography (OCT), OCT angiography (OCTA), adaptive optics (AO), and retinal function imaging (RFI), provide unprecedented insights into microvascular dynamics, structural changes, and functional impairments associated with ocular BD. Integration of these advanced imaging modalities enhances early detection of subclinical disease, facilitates precise localization of inflammatory lesions, guides therapeutic interventions, and monitors treatment efficacy. OCT and OCTA, in particular, offer non-invasive, high-resolution assessments of macular edema, vascular perfusion abnormalities, and choroidal thickness alterations critical for optimizing patient care. In conclusion, multimodal imaging represents the cornerstone in the comprehensive management of ocular manifestations in Behcet's Disease, offering clinicians invaluable tools for accurate diagnosis, treatment planning, and long-term monitoring of disease progression and treatment outcomes.

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Retinal vasculitis severity assessment: intra- and inter-observer reliability of a new scheme for grading wide-field fluorescein angiograms in retinal vasculitis

Cited by 3 publications

References 26 publications

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Using Deep Learning to Segment Retinal Vascular Leakage and Occlusion in Retinal Vasculitis

The Current and Novel Imaging Modalities for Ocular Vasculitis in Behcet’s Disease: A Review

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