Development of diagnostic criteria for Behçet's uveitis in a Chinese population

To develop and validate a novel set of ocular-centric diagnostic criteria for Behçet's uveitis (BU).

A case-control study was designed to develop the BU-specific diagnostic criteria (BU-SDC). The International Criteria for Behçet's Disease were adopted as the reference standard for diagnostic performance evaluation.

The ocular findings of BU-SDC were weighted as two points for vitreous cells or haze and retinal vasculitis identified by fluorescein fundus angiography, respectively, and one point for anterior cell-flare dissociation, sterile hypopyon, diffuse retinal atrophy, optic nerve atrophy, superficial retinal infiltrates, retinal haemorrhages, retinal vascular sheathing and retinal ghost vessels, respectively. The systemic features, including recurrent oral ulceration, multiform skin lesions and genital ulceration were weighted as 4 points, 3 points and 2 points, respectively. Primary assessment was based on ocular features alone. An ocular score ≥5 supported a BU diagnosis in patients without signs of granulomatous uveitis or evidence of sarcoidosis or syphilis. For patients with ocular score <5 but high clinical suspicion (the presence of one or more characteristic ocular signs, including vitreous cells or haze, retinal vasculitis identified by fluorescein fundus angiography, superficial retinal infiltrates or retinal vascular sheathing), a secondary assessment incorporating systemic variables was applied, and a combined score ≥5 supported diagnosis. Validation in the independent cohort showed that the area under the receiver operating characteristic curve for BU-SDC (0.954) significantly outperformed the International Study Group criteria (0.911; p<0.01) and the Standardization of Uveitis Nomenclature (0.881; p<0.01) criteria.

The BU-SDC provides a validated ocular-centric diagnostic framework for BU, prioritising ocular signs while retaining flexibility through selective systemic integration.