Vogt-小柳-原田病复发的临床特征与风险因素及治疗进展

Clinical features, risk factors and progresses on treatment of recurrent Vogt-Koyanagi-Harada disease

Vogt-小柳-原田病（VKH）是一种伴有神经系统、内耳及皮肤改变的双眼肉芽肿性全葡萄膜炎。临床上早期积极的糖皮质激素治疗一般可使病情得到较快控制，甚至治愈，不出现明显并发症。但部分患者炎性反应复发，并可能因并发性白内障、继发性青光眼、黄斑病变等并发症导致视力出现严重损伤。VKH复发以前葡萄膜炎表现居多，伴脉络膜增厚。VKH复发的可能风险因素包括发病初期视力差、前房炎性反应重、脉络膜皱褶、全身糖皮质激素用药减量过快或疗程不足、伴发眼外表现等。足疗程的糖皮质激素用药是治疗VKH复发的有效控制策略，而免疫抑制剂和生物制剂在治疗VKH慢性复发和难治性VKH方面的积极作用逐渐受到重视。本文将对相关研究进行总结，以期为临床诊疗工作提供依据。

Vogt-Koyanagi-Harada disease（VKH） is a bilateral, granulomatous panuveitis associated with central nervous system, auditory, and integumentary manifestations. Clinically, VKH usually responds well to early aggressive glucocorticosteroid treatment and may be cured without any clinically significant sequelae. Some patients, however, may enter the chronic recurrent phase, which may result in marked loss of vision due to complications such as complicated cataract, secondary glaucoma and maculopathy. Recurrent VKH is mainly characterized by anterior uveitis associated with thickening of the choroid. Initial poor visual acuity, severe anterior chamber reaction, choroidal folds,rapid tapering of systemic corticosteroids or inadequate duration of treatment, and development of extraoeular manifestations may be risk factors of disease recurrence. Prolonged glucocortieosteroid treatment has been suggested as effective strategy for recurrence of VKH. The positive effects of other immunosuppressive agents and biologic agents on treatment of chronic recurrent and refractory VKH have been gradually recognized by the uveitis community.