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PURPOSE To evaluate if the presence of uveitis in Behçet's disease (BD) is associated with a particular clinical phenotype and to analyze the prognostic impact of a missed diagnosis of BD at the time the uveitis is diagnosed. MATERIEL AND METHODS Ophthalmologic and systemic clinical features of 51 patients with BD were recorded retrospectively. We compared the clinical phenotype of patients with ocular manifestations with those without ocular manifestations. The patients were divided into two groups depending on the progression of their visual acuity: "decreased visual acuity" versus "stable or improved visual acuity." RESULTS In the group of patients with ocular involvement, there was a mean 2.3 systemic manifestations, vs. 3.2 in the group without ocular manifestations (P=0.004). When BD was diagnosed prior to the onset of uveitis, we counted fewer patients in the "decreased visual acuity" group in comparison with the patients who had no prior diagnosis of BD at the onset of the uveitis (91.3% in the "decreased visual acuity" group, P=0.04). The time before initiation of immunosuppressive treatment or a biological agent was shorter for these patients (4.4 vs. 39.3 months, P=0.007). CONCLUSION It appears that different phenotypes exist according to whether or not the BD patient has ocular involvement. Moreover, the visual prognosis is better if the uveitis occurs in patients who have already been diagnosed with BD, due to earlier initiation of immunosuppressive therapy.