Bonnet C, Daudin J-B, Monnet D, Brézin A
Université Paris Descartes, 75014 Paris, France; Centre Cochin ambulatoire d'ophtalmologie, groupe hospitalier Cochin-Hôtel-Dieu, 27, rue du Faubourg-Saint-Jacques, 75014 Paris, France.
Centre Cochin ambulatoire d'ophtalmologie, groupe hospitalier Cochin-Hôtel-Dieu, 27, rue du Faubourg-Saint-Jacques, 75014 Paris, France.
J Fr Ophtalmol. 2017 Jun;40(6):512-519. doi: 10.1016/j.jfo.2017.02.006. Epub 2017 Jun 1.
Vogt-Koyanagi-Harada (VKH) disease is defined as a severe bilateral, chronic granulomatous panuveitis associated with serous retinal detachments, disk edema, and vitritis, with central nervous system, auditory, and integumentary manifestations. It is an autoimmune inflammatory condition mediated by T cells that target melanocytes in individuals genetically susceptible to the disease. Vogt-Koyanagi-Harada disease presents clinically in 4 different phases: prodromal, acute inflammatory, chronic, and recurrent, with extraocular manifestations including headache, meningitis, hearing loss, poliosis, and vitiligo. Optical coherence tomography (OCT) allows earlier diagnosis of VKH disease by revealing heterogeneous exudative detachments of the retina in the acute stage and choroidal thickening, and by demonstrating choroidal thinning in the chronic stage. Treatment of this disease is initially with intravenous corticosteroids, with, if needed, a transition to immunosuppressant drugs for long-term control. Patients with VKH disease can have good final visual outcomes if treated promptly and aggressively.
伏格特-小柳-原田(VKH)病被定义为一种严重的双侧慢性肉芽肿性全葡萄膜炎,伴有浆液性视网膜脱离、视盘水肿和玻璃体炎,并伴有中枢神经系统、听觉和皮肤表现。它是一种由T细胞介导的自身免疫性炎症疾病,T细胞靶向对该疾病具有遗传易感性个体的黑素细胞。伏格特-小柳-原田病临床上呈现4个不同阶段:前驱期、急性炎症期、慢性期和复发期,眼外表现包括头痛、脑膜炎、听力丧失、白发症和白癜风。光学相干断层扫描(OCT)通过在急性期显示视网膜的异质性渗出性脱离和脉络膜增厚,以及在慢性期显示脉络膜变薄,从而实现对VKH病的早期诊断。该疾病的治疗最初采用静脉注射皮质类固醇,如有需要,可过渡到使用免疫抑制药物进行长期控制。如果及时且积极地进行治疗,VKH病患者可获得良好的最终视力结果。