Bonfioli Adriana A, Orefice Fernando
Eye & Ear Institute of Pittsburgh, University of Pittsburgh Medical Center, Pittsburgh, PA, USA.
Semin Ophthalmol. 2005 Jul-Sep;20(3):199-206. doi: 10.1080/08820530500231953.
Behçet's disease is a chronic, relapsing vasculitis that can affect most organ systems. The prevalence varies geographically, and the disease is more common in countries along the ancient Silk Road, including Italy, Turkey, Israel, Saudi Arabia, Iran, China, Korea and Japan. Behçet's is more common in men than in women, and typically affects young adults. The classic finding in Behçet's patients is the presence of recurrent mucocutaneous ulcers, and oral aphthous ulcerations are usually the initial symptom. Other manifestations include genital ulcers, skin lesions, vascular, neurological, articular, and ocular disease. The disease can affect the anterior and/or posterior segments of the eye, and the main manifestations include iridocyclitis, hypopyon, mild to moderate vitreitis, retinal vasculitis and occlusion, optic disc hyperemia, and macular edema. There is no pathognomonic laboratory test in Behçet's disease, and the diagnosis is based in systemic and ocular clinical findings. Treatment of ocular Behçet is based in corticosteroids and immunosuppressive agents, to suppress acute inflammation and reduce its recurrence frequency. Ocular lesions may improve with immunosuppressive therapy, but usually are not fully reversible, and generally progress over time. The prognosis of anterior uveitis is usually good, but patients with posterior lesions tend to have some degree of visual loss, even with adequate treatment.
白塞病是一种可累及多数器官系统的慢性复发性血管炎。其患病率存在地域差异，在古代丝绸之路沿线国家更为常见，包括意大利、土耳其、以色列、沙特阿拉伯、伊朗、中国、韩国和日本。白塞病在男性中比在女性中更常见，且通常影响年轻成年人。白塞病患者的典型表现是反复出现黏膜皮肤溃疡，口腔阿弗他溃疡通常是初始症状。其他表现包括生殖器溃疡、皮肤病变、血管病变、神经病变、关节病变和眼部疾病。该病可累及眼球前段和/或后段，主要表现包括虹膜睫状体炎、前房积脓、轻至中度玻璃体炎、视网膜血管炎和闭塞、视盘充血以及黄斑水肿。白塞病没有特异性的实验室检查，诊断基于全身和眼部临床发现。眼部白塞病的治疗以使用糖皮质激素和免疫抑制剂为主，以抑制急性炎症并降低复发频率。免疫抑制治疗可能会使眼部病变有所改善，但通常无法完全逆转，且一般会随时间进展。前葡萄膜炎的预后通常良好，但即使接受充分治疗，后段病变患者仍往往会有一定程度的视力丧失。
