Vogt-小柳-原田病的临床特点和治疗分析

目的探讨Vogt-小柳-原田病(Vogt-Koyanagi-Harada,VKH)的临床特点、治疗和预后等问题。方法连续收集近两年间在我院就诊的VKH患者45例,对其眼部表现、全身症状、荧光素眼底血管造影(fundus fluo-rescein angiography,FFA)和治疗进行回顾性分析。结果45例患者均为双眼发病,男女比例是1:1.4,平均年龄39.3岁。发病前有前驱症状者37例(82.2%),主要为头痛。就诊时有眼外症状和体征者39例(86.7%),包括中枢神经系统异常、听力障碍、白发脱发和白癜风。发病后2周内就诊者11例,表现为双眼脉络膜炎、视盘及视网膜水肿;发病后2周至3个月内就诊者24例,主要表现为非肉芽肿性前葡萄膜炎、后葡萄膜炎和浆液性视网膜脱离;复发患者(病程3个月以上)10例,主要表现为肉芽肿性前葡萄膜炎、晚霞状眼底改变和Dalen-Fuchs结节。FFA检查表现为斑驳状高荧光、视盘染色和多湖样染料积存。所有患者给予大剂量糖皮质激素治疗,疗程为3～18个月。初发组94.3%患者葡萄膜炎完全控制,视力显著提高。10例复发者中炎症完全控制7例(70%),视力有不同程度提高。结论前驱症状、临床病程、眼外表现及FFA等有助于VKH的诊断,早期给予大剂量糖皮质激素治疗,绝大部分患者可控制炎症,改善视力。

Clinical manifestations and treatment of Vogt-Koyanagi-Harada disease

Objective To investigate the clinical features, treatment and prognosis of Vogt-Koyanagi-Harada (VKH) disease. Methods The data of 45 patients with VKH disease in Tianjin Eye Hospital from June 2004 to June 2006 were analyzed retrospectively. Patients were carefully examined with ocular presentation, systemic symptom and fundus fluorescein angiography (FFA). Results Simultaneous involvement of both eyes occurred in all of these patients. The ratio of male to female was 1:1.4. The mean age was 39.3. There were 37 patients(82.2%) with prodrome, and the headache was the most common. Extraocular manifestations were noted in 86.7% of the patients, including neurologic symptoms, auditory problems, poliosis, alopecia and vitiligo. Choroiditis, papilledema and edema of the retina were noted in 11 patients within 2 weeks of disease onset. Non-granulomatous anterior uveitis, posterior uveitis and exudative retinal detachment were noted in 24 patients from 2 weeks to 3 months of disease onset. Granulomatous anterior uveitis, "sunset glow" fundus and Dalen-Fuchs nodules were noted in 10 recurrent patients over 3 months of disease onset. The typical findings of FFA were irregular punctate hyperfluorescent dots, disc leakage and multiple hyperfluorescent pool in the subretina. All patients were treated with high-dose corticosteroids for 3~18 months. Uveitis was completely controlled in 94.3% of the patients with first attack, and all of these patients showed improved vision after treatment. Uveitis was completely controlled in 70% of the patients with recurrent episodes, and vision improved differently. Conclusion Prodrome, clinical course, extraocular manifestations and FFA are helpful to the diagnosis of VKH disease. Early and aggressive systemic treatment with corticosteroids has greatly improved the visual outcome in VKH patients.