The typical clinical findings of Behcet disease are complex, including oral ulcer, vulvar ulcer, skin rash, erythema nodosum, uveitis, esophageal ulcer, colon ulcer, joint pain, etc. The diagnosis depends on typical clinical findings, without specific laboratory findings. Increase in CRP and ESR are probably the only laboratory findings. The etiology and pathogenesis of this disease is not fully understood. Vasculitis is the foremost histopathological lesion affecting various blood vessels in Behcet disease. According to vessel involvement, Behcet disease has demonstrated 3 main clinical features: venous occlusion, arterial occlusion, and aneurysm formation.[6,7] Endothelial dysfunction leaded by vascular inflammation is thought to be a major factor for thrombosis. Thrombosis is the most important cause of vascular occlusion. Some studies have suggested that risk factors for thrombosis include increased endothelial activation markers, defective fibrinolysis, and/or impaired coagulation, reflect vascular cell injury, inherited thrombophilia, demographic, genes, immune system, race, HLA-B5 antigen, etc.[9,10]
Uveitis in patients with Behcet disease can affect the anterior segment, posterior, or peripheral retina. Patients with involvement of anterior segment usually develop nongranulomatous uveitis, with anterior chamber empyema being the most common. Meanwhile, patients with involvement of posterior segment usually develop vitreous opacity, retinal invasion, vascular sheaths, vascular occlusion, macular edema, and other inflammatory manifestations.
For ocular lesions caused by Behcet disease, in addition to eye topical anti-inflammatory symptomatic treatment, corticosteroids are the most widely used systemic treatment, which combined with immunosuppressive agents can achieve better curative effect in patients with severe first episode or repeated relapse, and help to reduce drug side effects with a combination of glucocorticoids. Biological agents (such as tumor necrosis factor-blockers and alpha-interferon), which have been used in recent years, are expensive, and have several side effects and high relapse rates after discontinuation. Their safety, efficacy, and cost-effectiveness need further evaluation.[11,12]
Behcet disease has unclear etiology, and lacks characteristic laboratory findings. Its diagnosis is mainly based on clinical manifestations, and more positive signs lead to more reliable diagnosis. Hence, ophthalmologists should not exclude this systemic disease by solely relying on the laboratory findings of patients with uveitis. Missed diagnosis of Behcet disease is likely if the general clinical manifestations are ignored, thereby missing the optimal timing of treatment. Patients with Behcet disease are susceptible to various types of ocular inflammation with different manifestations. However, the patient in this study suffered from vascular occlusion and sudden vision loss because his unilateral retinal vasculitis affected both the central retinal artery and vein, which is very rare, and shows very poor curative effect and prognosis. This case also cautions ophthalmologists of the high possibility of vasculitis (such as Behcet disease) in young and middle-aged patients with retinal vascular occlusion. Hence, the ophthalmologists should investigate systemic diseases, and obtain detailed medical history. This case further suggests that Behcet disease has diverse manifestations, complex diagnosis, and severe lesions, and the recurrence rate must be controlled to minimize irreversible consequences such as sudden vision loss during recurrence.
Written informed consent was obtained for the publication of the case details.
The authors want to thank Ms Xiaoyue Wang and Mr Zhibing Zeng in Imaging Department, Sichuan University West China Hospital for the excellent work on fundus photography and fluorescein fundus angiography.
Conceptualization: Yunxia Gao, Ming Zhang.
Data curation: Yunxia Gao, Shuyuan Lyu, Li Bao.
Formal analysis: Yunxia Gao, Shuyuan Lyu.
Investigation: Yunxia Gao, Shuyuan Lyu, Ming Zhang.
Methodology: Yunxia Gao.
Project administration: Yunxia Gao, Li Bao, Ming Zhang.
Resources: Yunxia Gao, Li Bao, Ming Zhang.
Supervision: Yunxia Gao, Shuyuan Lyu, Ming Zhang.
Writing – original draft: Yunxia Gao.
Writing – review and editing: Yunxia Gao, Shuyuan Lyu, Ming Zhang.
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Keywords:Copyright © 2018 The Authors. Published by Wolters Kluwer Health, Inc. All rights reserved.
Behcet disease; central retinal artery occlusion; central retinal vein occlusion; diagnosis; therapy; uveitis; vasculitis