Vogt-Koyanagi-Harada综合征的临床表现及诊断

目的 探讨Vogt-Koyanagi-Harada(VKH)综合征患者的临床特征.盲目原因及诊断等有关问题.方法 对在1996年1月至2000年12月间就诊资料完整的157例VKH综合征患者进行回顾性分析,并对裂隙灯,眼底镜,三面镜,荧光素眼底血管造影(fundus fluorescein angiography,FFA),吲哚青绿血管造影(indocyanine green angiography,ICGA)及人类白细胞抗原(HLA)分型等检查结果进行分析.结果 VKH综合征最常见的前驱症状为头痛(102例,73.5%),双眼同时患病118例(80.8%);后葡萄膜炎期眼部主要表现为脉络膜炎,视乳头及附近视网膜水肿(100.0%);前葡萄膜炎反复发作期眼部表现为复发性肉芽肿性前葡萄膜炎(128例,98.4%),晚霞状眼底改变(95.8%)及Dalen-Fuchs结节(71.2%),后葡萄膜炎期,前葡萄膜受累期及前葡萄膜炎反复发作期导致盲目的主要原因分别为视乳头炎,视网膜脱离及并发性白内障;毛发变白(36.3%)及脱发(35.0%)是最常见的眼外表现;炎症活动期FFA典型表现为斑驳状高荧光,ICGA发现脉络膜血管扩张,通透性增高等改变;VKH综合征患者HLA-DR4及HLA-DRw53的阳性率(54.9%及71.8%)显著高于正常对照组(14.7%及38.2%).结论 VKH综合征患者在后葡萄膜炎期眼部典型表现为双侧脉络膜炎,视乳头炎或神经视网膜炎,随后出现以反复发作的肉芽肿性前葡萄膜炎为特征的全葡萄膜炎.眼外症状及相关的辅助检查包括FFA,ICGA及HLA分型等有助于VKH综合征的诊断。

Clinical manifestations and diagnosis of Vogt-Koyanagi-Harada syndrome

Objective To investigate the clinical features, causes of blindness and diagnosis of Vogt Koyanagi Harada (VKH) syndrome Methods The data of 157 patients with VKH syndrome were reviewed and analyzed Patients were carefully examined with slit lamp, ophthalmoscope, three mirror lens, fundus fluorescein angiography (FFA), indocyanine green angiography (ICGA) and HLA typing Results Headache was noted in 73 5% of these patients Simultaneous involvement of both eyes occurred in 80 8% of these patients Chroiditis,papilledema and edema of the retina adjacent to the optic nerve were noted in 100% of these patients in the posterior uveitis stage, whereas recurrent granulomatous anterior uveitis (98 4%), "sunset glow" fundus (95 8%) and Dalen Fuchs nodules (71 2%) were the common ocular findings in the recurrent anterior uveitis stage The common causes of blindness were papillitis, exudative retinal detachment and complicated cataract in the posterior uveitis stage, anterior uveal involvement stage and its recurrent stage Poliosis (36 3%) and alopecia (35 0%) were the most common extraocular findings Early irregular patches of fluorescence, followed by localized hyperfluorescent spots were the typical findings of FFA Dilation of choroidal vessels and leakage of ICG from the choroidal vessels were the common ICGA findings The prevalence of HLA DR4 and HLA DRw53 in patients (54 9% and 71 8% respectively) was significantly higher than that in controls (14 7% and 38 2% respectively) Conclusions VKH syndrome is characterized by chroiditis, papillitis or neuroretinitis in the posterior uveitis stage, followed by a generalized uveitis with a typical recurrent granulomatous anterior uveitis Extraocular findings and relevant examinations including FFA, ICGA and HLA typing are helpful to the diagnosis of VKH syndrome