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Objectives: To analyze and compare sociodemographic features between Behçet uveitis and other non-infectious uveitis. Materials and Methods: The data of adults with non-infectious uveitis in the nationwide uveitis database were analyzed and the sociodemographic features of patients with and without Behçet disease were compared. Results: This study included data of 4,978 eyes of 3,363 patients from 33 centers. The mean age at presentation was 38.7±13.3 (17- 87) years. The mean age was 34.3±10.5 years in the Behçet uveitis group and 41.1±14.0 years in the other non-infectious uveitis group (p<0.001). Male predominance was seen in the Behçet uveitis group (67.7% vs. 32.3%) while female patients were more common in the other non-infectious uveitis group (54.4% vs. 45.6%, p<0.001). Regarding education level, the proportion of patients with low education was higher in the Behçet uveitis group than the other non-infectious uveitis group (49.6% vs. 43.4% in males, p=0.004; 61.5% vs. 59.2% in females, p=0.021). Having a low-income job or being currently unemployed, indicators of poor income, were more frequent in the Behçet uveitis group than in the other non-infectious uveitis group (32.0% vs. 22.8%, p<0.001). In the comparison of places of residence, the proportion of patients who lived in cities with low gross national product was 37.0% in the Behçet uveitis group and 31.1% in the other non-infectious uveitis group (p<0.001). Conclusion: Patients with Behçet disease had lower education level and socioeconomic status than patients with other non-infectious uveitis entities. [ABSTRACT FROM AUTHOR]

Genome-wide association studies (GWAS) are a powerful means of identifying genes with disease-associated common variants, but they are not well-suited to detecting genes with disease-associated rare and low-frequency variants. In the current study of Behçet disease (BD), nonsynonymous variants (NSVs) identified by deep exonic resequencing of 10 genes found by GWAS (IL10, IL23R, CCR1, STAT4, KLRK1, KLRC1, KLRC2, KLRC3, KLRC4, and ERAP1) and 11 genes selected for their role in innate immunity (IL1B, IL1R1, IL1RN, NLRP3, MEFV, TNFRSF1A, PSTPIP1, CASP1, PYCARD, NOD2, and TLR4) were evaluated for BD association. A differential distribution of the rare and low-frequency NSVs of a gene in 2,461 BD cases compared with 2,458 controls indicated their collective association with disease. By stringent criteria requiring at least a single burden test with study-wide significance and a corroborating test with at least nominal significance, rare and low-frequency NSVs in one GWAS-identified gene, IL23R (P = 6.9 × 10-5), and one gene involved in innate immunity, TLR4 (P = 8.0 × 10-4), were associated with BD. In addition, damaging or rare damaging NOD2 variants were nominally significant across all three burden tests applied (P = 0.0063-0.045). Furthermore, carriage of the familial Mediterranean fever gene (MEFV) mutation Met694Val, which is known to cause recessively inherited familial Mediterranean fever, conferred BD risk in the Turkish population (OR, 2.65; P = 1.8 × 10-12). The disease-associated NSVs in MEFV and TLR4 implicate innate immune and bacterial sensing mechanisms in BD pathogenesis. [ABSTRACT FROM AUTHOR]

Behçet's disease is a multisystem inflammatory disorder that is most common in countries along the ancient "Silk Road". The eye is the most commonly involved vital organ in Behçet's patients and the typical form of involvement is a relapsing remitting panuveitis and retinal vasculitis. Uveitis is the initial manifestation of the disease in 10-15% of the patients. Anterior uveitis is always nongranulomatous. Diffuse vitritis, retinal infiltrates, sheathing of predominantly retinal veins, and occlusive vasculitis are the typical signs of posterior segment inflammation. Spontaneous resolution of acute inflammatory signs is a diagnostic feature. Fundus fluorescein angiography is the gold standard in monitoring inflammatory activity. Laser flare photometry is a useful noninvasive tool since flare readings correlate with fluorescein angiographic leakage. The most common complications are cataract, maculopathy, and optic atrophy. Male patients have a more severe disease course and worse visual prognosis. Immunomodulatory therapy is indicated in all patients with posterior segment involvement. Corticosteroids combined with azathioprine and/or cyclosporine is used initially. Biologic agents, including interferon alfa and infliximab, are used in resistant cases. Visual prognosis has improved in recent years with an earlier and more aggressive use of immunomodulatory therapy and the use of biologic agents in resistant cases. [ABSTRACT FROM AUTHOR]