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[伏格特-小柳-原田综合征(临床病例)]

[Vogt-Koyanagi-Harada syndrome (clinical cases)].

作者信息

Sorokin E L, Voronina N V, Avramenko S Yu, Pomytkina N V

机构信息

Khabarovsk branch of the Academician S.N. Fyodorov IRTC 'Eye Microsurgery', 211 Tikhookeanskaya St., Khabarovsk, Russian Federation, 680033; Far Eastern State Medical University, 35 Muravyeva-Amurskogo St., Khabarovsk, Russian Federation, 680000.

Far Eastern State Medical University, 35 Muravyeva-Amurskogo St., Khabarovsk, Russian Federation, 680000.

出版信息

Vestn Oftalmol. 2015 May-Jun;131(3):90-98. doi: 10.17116/oftalma2015131390-96.

Abstract

OBJECTIVE

to analyze two female cases of Vogt-Koyanagi-Harada (VKH) syndrome.

MATERIAL AND METHODS

The first patient presented with bilateral panuveitis and unilateral keratomalacia (left eye). For the latter, blepharorrhaphy was performed. Methylprednisolone (Metypred) and azathioprine pulses, subsequently switched to oral therapy, caused regression of uveitis. In 1 month the patient was operated for retinal detachment and associated cataract in her right eye. The second patient presented with bilateral detachment of neuroepithelium. Complete reattachment in both eyes was achieved with Metypred pulses followed by oral prednisolone and azathioprine. Bilateral panuveitis with pupil occlusion developed 6 months after the cessation of prednisolone, however, began to resolve as soon as the treatment was resumed.

RESULTS

Timely diagnosis and combination pulse therapy (methylprednisolone and azathioprine) enabled rapid resolution of acute condition.

CONCLUSIONS

Pulse Metypred is the mainstay of the treatment of VKH syndrome. Supplementing the treatment of autoimmune uveitis with low doses of azathioprine slows progression of the disease and minimizes prednisolone-induced side effects. It is important that VKH patients are treated with pathogenetic therapy in close cooperation with an immunologist. Long-term monitoring (not less than 2-3 years) is also necessary.

摘要

目的

分析两例Vogt-小柳-原田(VKH)综合征女性病例。

材料与方法

首例患者表现为双侧全葡萄膜炎和单侧角膜软化(左眼)。针对角膜软化,实施了睑缘缝合术。静脉注射甲泼尼龙(美卓乐)和硫唑嘌呤,随后改为口服治疗,葡萄膜炎症状消退。1个月后,该患者右眼因视网膜脱离及并发白内障接受了手术。第二例患者表现为双侧神经上皮脱离。通过静脉注射甲泼尼龙,随后口服泼尼松龙和硫唑嘌呤,双眼实现了完全复位。停用泼尼松龙6个月后,出现了双侧全葡萄膜炎伴瞳孔闭锁,但治疗恢复后症状即开始缓解。

结果

及时诊断并采用联合脉冲疗法(甲泼尼龙和硫唑嘌呤)可使急性症状迅速缓解。

结论

静脉注射甲泼尼龙是VKH综合征治疗的主要手段。低剂量硫唑嘌呤辅助自身免疫性葡萄膜炎治疗可减缓疾病进展,并将泼尼松龙所致副作用降至最低。VKH患者与免疫学家密切合作进行病因治疗非常重要。长期监测(不少于2至3年)也是必要的。

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