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Background: Behçet's disease (BD) is a relapsing-remitting vasculitis, which can manifest in different organ systems including the eyes. There is currently limited published data describing the incidence of ophthalmic disease within the United Kingdom. The primary aim of this study was to survey the incidence and manifestations of ophthalmic BD prospectively, with a secondary aim of reviewing treatment modalities initiated in first-line therapy., Methods: Using the British Ophthalmic Surveillance Unit reporting system between October 2016 and November 2018, we prospectively surveyed the number of cases of BD presenting to UK ophthalmologists. A total of 89 cases of ophthalmic manifestations of BD were reported and complete information was collected on 58 patients., Results: 93 eyes of 58 patients were affected. The median age of reported cases was 31 years (range 13-55 years) who were born in 15 different countries. Most cases (n = 35, 60%) had bilateral involvement. Vitritis was the most common ocular manifestation (68%; n = 63) followed by anterior uveitis (46%; n = 43). The greatest causes of visual morbidity were cystoid macular oedema, vitritis and retinal ischaemia. Most patients were prescribed either topical or oral corticosteroids (59%; n = 34), with some given intravitreal or intravenous corticosteroids. Five patients (8.6%) were initiated on disease-modifying anti-rheumatic drugs and one given an anti-TNF monoclonal antibody., Conclusions: This is the first prospective study to analyse the incidence of ophthalmic involvement in BD over a 2-year period, finding an annual incidence of 0.04 per 100,000 individuals in the UK., (© 2021. The Author(s), under exclusive licence to The Royal College of Ophthalmologists.)

Purpose : To develop an algorithm for the diagnosis of Behçet's disease (BD) uveitis based on ocular findings. Methods : Following an initial survey among uveitis experts, we collected multi-center retrospective data on 211 patients with BD uveitis and 207 patients with other uveitides, and identified ocular findings with a high diagnostic odds ratio (DOR). Subsequently, we collected multi-center prospective data on 127 patients with BD uveitis and 322 controls and developed a diagnostic algorithm using Classification and Regression Tree (CART) analysis and expert opinion. Results : We identified 10 items with DOR >5. The items that provided the highest accuracy in CART analysis included superficial retinal infiltrate, signs of occlusive retinal vasculitis, and diffuse retinal capillary leakage as well as the absence of granulomatous anterior uveitis or choroiditis in patients with vitritis. Conclusion : This study provides a diagnostic tree for BD uveitis that needs to be validated in future studies.