Herbort Carl P, Abu El Asrar Ahmed M, Takeuchi Masuru, Pavésio Carlos E, Couto Cristobal, Hedayatfar Alireza, Maruyama Kazuichi, Rao Xi, Silpa-Archa Sukhum, Somkijrungroj Thanapong
Retinal and Inflammatory Eye Diseases, Centre for Ophthalmic Specialized Care (COS), Clinic Montchoisi Teaching Centre, Rue Charles-Monnard 6, 1003, Lausanne, Switzerland.
Department of Ophthalmology, University of Lausanne, Lausanne, Switzerland.
Int Ophthalmol. 2019 Jun;39(6):1419-1425. doi: 10.1007/s10792-018-0949-4. Epub 2018 Jun 11.
Vogt-Koyanagi-Harada (VKH) disease is a primary autoimmune granulomatous choroiditis that begins in the choroidal stroma. The aim of this review was to gather a body of evidence for the concept of a window of therapeutic opportunity, defined as a time interval following initial-onset disease during which adequate treatment will substantially modify the disease outcome and possibly even lead to cure, similar to what has been described for rheumatoid arthritis.
We reviewed the literature and consulted leading experts in VKH disease to determine the consensus for the notion of a therapeutic window of opportunity in VKH disease.
We found a substantial body of evidence in the literature that a therapeutic window of opportunity exists for initial-onset acute uveitis associated with VKH disease. The disease outcome can be substantially improved if dual systemic steroidal and non-steroidal immunosuppressants are given within 2-3 weeks of the onset of initial VKH disease, avoiding evolution to chronic disease and development of "sunset glow fundus." Several studies additionally report series in which the disease could be cured, using such an approach.
There is substantial evidence for a therapeutic window of opportunity in initial-onset acute VKH disease. Timely and adequate treatment led to substantial improvement of disease outcome and prevented chronic evolution and "sunset glow fundus," and very early treatment led to the cure after discontinuation of therapy in several series, likely due to the fact that the choroid is the sole origin of inflammation in VKH disease.
伏格特-小柳-原田（VKH）病是一种始于脉络膜基质的原发性自身免疫性肉芽肿性脉络膜炎。本综述的目的是收集证据，以支持治疗机会窗这一概念，该窗被定义为疾病初发后的一个时间间隔，在此期间进行充分治疗将显著改变疾病结局，甚至可能治愈，这与类风湿关节炎的情况类似。
我们查阅了文献，并咨询了VKH病领域的顶尖专家，以确定VKH病治疗机会窗概念的共识。
我们在文献中发现了大量证据，表明与VKH病相关的初发性急性葡萄膜炎存在治疗机会窗。如果在VKH病初发后2 - 3周内给予全身性甾体和非甾体免疫抑制剂，可显著改善疾病结局，避免发展为慢性病和出现“晚霞眼底”。多项研究还报告了使用这种方法使疾病得以治愈的系列病例。
有大量证据表明初发性急性VKH病存在治疗机会窗。及时且充分的治疗可显著改善疾病结局，预防慢性病发展和“晚霞眼底”，并且在一些系列病例中，极早期治疗在停药后实现了治愈，这可能是因为脉络膜是VKH病炎症的唯一起源。
