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Behçet disease (BD) is a multisystem inflammatory disorder that is an important cause of morbidity worldwide. BD is most common along the ancient "Silk Road" route in the Far East and Mediterranean basin. The eye is the most commonly involved organ in BD patients. The prototypical form of involvement is a relapsing remitting panuveitis and retinal vasculitis. Less commonly, BD may present in the form of conjunctivitis, conjunctival ulcers, keratitis, episcleritis, scleritis, and extraocular muscle paralysis. Uveitis in BD carries significant implications for the patient, because it is a chronic recurrent disease characterized by explosive attacks of severe inflammation that may cause significant, cumulative damage to the intraocular structures. This review summarizes the epidemiology of systemic and ocular clinical features of BD with particular focus on risk factors, clinical characteristics, complications, and prognosis of BD-associated uveitis. [ABSTRACT FROM AUTHOR]

The author reflects on Behcet Uveitis (BU). Topics discussed include Behcet disease (BD) as a systemic disorder; and ocular complications of BU that are edema, non-perfusion, neovascularization, and atrophy of the retina and optic disc. Also being discussed are tumor necrosis factor (TNF) inhibitor or interferon, conventional immunosuppressive therapy, and indocyanine green angiography (ICGA) and fundus autofluroescence (FAF).

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. [ABSTRACT FROM AUTHOR]

Objectives: To investigate the changes over time in extraocular and ocular manifestations of Behçet's disease (BD) in Tunisian patients. Material and methods: Retrospective study of 246 patients divided into two groups: group 1 (147 patients examined from 1995 to 2005) and group 2 (99 patients examined from 2006 to 2017). Results: Active or scarred genital ulcers observed by physician at presentation were significantly less frequent in group 2 (47.2% vs. 29.6%; p = 0.007), as were articular involvement (50.3% vs. 34.7%; p = 0.016) and erythema nodosum (18.4% vs. 8.1%; p = 0.024). One hundred-seven patients (43.5%) developed ocular manifestations during the 23-year study period. Intermediate uveitis was significantly more frequent in group 2 than in group 1 (11.7% vs. 28.4%; p = 0.003), and posterior uveitis less frequent in group 2 than in group 1 (34.2% vs. 19.7%; p = 0.016). Patients from group 2 were more likely to have macular edema (19.8% vs. 45.6%; p = 0.001). However, better visual prognosis, with a lower rate of legal blindness, was noted in group 2. Conclusions: Changes over time included a decrease in the rate of articular involvement and cutaneous involvement. There was an increase in the rate of intermediate uveitis and a decrease in the rate of posterior uveitis over time. Despite an increase in the rate of macular edema, there was an improvement in visual prognosis, with less legal blindness over time. [ABSTRACT FROM AUTHOR]

Purpose: To evaluate the safety and efficacy of gevokizumab for the treatment of Behçet's disease uveitis in a prospective, open-label, randomized phase 2 trial.Methods: Behçet's disease patients with new acute ocular exacerbation or at risk of exacerbation received 30 or 60 mg gevokizumab every 4 weeks intravenously or subcutaneously, on top of a stable regimen of immunosuppressives and corticosteroids (≤20 mg/day equivalent prednisolone). Patients withdrew in cases of ocular exacerbation.Results: A total of 21 patients were included (17 acute and 4 at-risk; mean duration of uveitis 45.6 ± 37.4 months). There were no serious adverse events related to gevokizumab. Recorded adverse events were mostly associated with exacerbation of uveitis or its complications. Response was evaluated for 14 acute patients and all showed rapid control of acute ocular exacerbation, mostly within 1 week, without any increase in corticosteroid dosage.Conclusions: Gevokizumab was well tolerated and rapidly controlled acute ocular exacerbations of Behçet's disease uveitis without the need for high-dose corticosteroid. [ABSTRACT FROM AUTHOR]

Behçet disease (BD) is a common, complex, and severely debilitating systemic vasculitis. Despite an increasing understanding of BD overall, there remain many critical questions to be addressed in terms of the epidemiology, pathophysiology, and treatment of this condition. The opinions of uveitis experts related to these issues were recently surveyed and are summarized herein. The survey results revealed significant disparities between responders in terms of important concepts regarding the clinical features, diagnosis, and treatment of this important inflammatory eye disease. [ABSTRACT FROM AUTHOR]

The aim of this retrospective study is to assess the frequency of HLA-B27 and HLA-B51 in healthy subjects from the center of Tunisia and to investigate their usefulness in the diagnosis of ankylosing spondylitis (AS) and Behçet's disease (BD), respectively. Microlymphocytotoxicity test was used to perform serologic HLA typing in a group of 124 healthy volunteers and a group of 365 patients suffering from clinical manifestations of AS and/or BD. HLA-B27 was found in 3.2% of healthy subjects and in 42.9% of patients with AS ( P < 0.00006). HLA-B51 is, however, found in 16.1% of healthy subjects and in 30.0% of patients with BD ( P > 0.05). Unlike HLA-B51, which seems to be as frequent in Tunisian patients with BD as in healthy subjects, HLA-B27 is more frequent in patients with AS than in controls. This highlights the usefulness of HLA-B27, rather than that of HLA-B51, in the diagnosis of the respective diseases. [ABSTRACT FROM AUTHOR]

Purpose: To report 2 cases of branch retinal artery occlusion (BRAO) associated with Behççet disease (BD). Design: Small case series. Methods: Review of two patients'' charts, fluorescein angiography, and optical coherence tomography (OCT). Results: The authors report two young patients, diagnosed with BD, who developed decreased vision secondary to BRAO for the first patient and cilioretinal artery occlusion associated with branch retinal vein occlusion for the second patient. In the two cases, artery occlusion was associated with active intraocular inflammation. Conclusions: BRAO, with subsequent transient or permanent visual impairment, is an additional ocular complication of BD. [ABSTRACT FROM AUTHOR]

Herein a case of a 35-year-old woman with a history of Behçet's disease, who presented with swelling and redness of her right eye with increasing pain, is reported. Computed tomography and magnetic resonance imaging showed enlargement of the right lacrimal gland and contrast enhancement of the extraocular muscles. A diagnosis of orbital inflammation was made and the patient was treated with corticosteroids, with prompt resolution of symptoms and clinical signs. Orbital inflammation should be considered as an ophthalmic manifestation of Behçet's disease. [ABSTRACT FROM AUTHOR]