Touitou V, Escande C, Bodaghi B, Cassoux N, Wechsler B, Lemaitre C, Tran T H C, Fardeau C, Piette J-C, LeHoang P
Service d'Ophtalmologie, CHU Pitié-Salpêtrière, 47-83, boulevard de l'Hôpital, 75651 Paris cedex 13, France.
J Fr Ophtalmol. 2005 Jan;28(1):9-16. doi: 10.1016/s0181-5512(05)81020-4.
To determine the most efficient diagnostic tools in Vogt-Koyanagi-Harada syndrome, taking into account the international diagnostic criteria, and to evaluate the therapeutic management of these patients.
This study examined patients with a suspicion of VKH syndrome who presented between January 2001 and March 2003, including ocular and extraocular evaluation of the disease at the time of diagnosis. Each patient was classified according to the 1978 international diagnostic criteria and the revised 2001 criteria. In most cases, intravenous steroid pulses were administered. Immunosuppressors were initiated when inflammation was not controlled with steroids.
Twenty-two patients were included. The mean age was 33.5 years (range, 15-49 years). Posterior segment involvement, which was observed in 21 patients, depended on the stage of the disease. Anterior segment inflammation was associated in eleven cases. Neurologic symptoms, including meningitis, cerebrospinal fluid lymphocytic pleocytosis, tinnitus, or hearing loss were observed in 12 patients. Fourteen patients had dermatologic signs. Five patients who developed VKH syndrome did not meet the 1978 criteria, and three patients did not meet the 2001 revised criteria. In 19 cases, intraocular inflammation was controlled with corticosteroids. In three cases, corticosteroids could not be discontinued. These patients were treated with immunosuppressive molecules: azathioprine, cyclophosphamide, interferon alpha. At the end of the follow-up period, inflammation was controlled in all patients.
Revision of the diagnostic criteria provides a more subtle diagnosis of VKH syndrome. However, it is difficult to consider the variability of clinical symptoms during the duration of disease. Corticosteroids must be used at appropriate dosages, followed by slow tapering over 6 months. This attitude seems to reduce the duration of ocular inflammation and decreases the frequency of recurrence. The use of immunomodulating drugs could be reduced by early and appropriate use of systemic steroids. Interferon alpha seems to be a promising alternative in corticoresistant or corticodependent forms of the disease, but further controlled studies are required.
考虑国际诊断标准，确定Vogt-小柳-原田综合征最有效的诊断工具，并评估这些患者的治疗管理。
本研究检查了2001年1月至2003年3月间疑似VKH综合征的患者，包括诊断时对该疾病的眼部和眼外评估。每位患者均根据1978年国际诊断标准和2001年修订标准进行分类。多数情况下，给予静脉注射类固醇脉冲治疗。当炎症未被类固醇控制时，开始使用免疫抑制剂。
纳入22例患者。平均年龄为33.5岁（范围15 - 49岁）。21例患者出现后段受累，这取决于疾病阶段。11例伴有前段炎症。12例患者出现神经系统症状，包括脑膜炎、脑脊液淋巴细胞增多、耳鸣或听力丧失。14例患者有皮肤体征。5例发生VKH综合征的患者不符合1978年标准，3例患者不符合2001年修订标准。19例患者的眼内炎症通过皮质类固醇得到控制。3例患者无法停用皮质类固醇。这些患者接受了免疫抑制药物治疗：硫唑嘌呤、环磷酰胺、干扰素α。随访期末，所有患者的炎症均得到控制。
诊断标准的修订为VKH综合征提供了更精确的诊断。然而，在疾病过程中考虑临床症状的变异性较为困难。必须以适当剂量使用皮质类固醇，随后在6个月内缓慢减量。这种做法似乎可缩短眼部炎症持续时间并降低复发频率。早期且适当地使用全身性类固醇可减少免疫调节药物的使用。干扰素α似乎是该疾病皮质抵抗或皮质依赖型的一种有前景的替代药物，但需要进一步的对照研究。
