Vogt-Koyanagi-Harada disease.
Vogt-Koyanagi-Harada disease.
复制标题
DOI：
10.1080/08820530500232126
复制
发表时间：
2005-07-01
影响因子：
1.7
通讯作者：
Young, Lucy H
中科院分区：
文献类型：
--
作者：
Damico, Francisco Max;Kiss, Szilard;Young, Lucy H
Vogt-Koyanagi-Harada病（VKH）是一种多系统自身免疫性疾病，主要影响眼、听觉、皮肤和中枢神经系统的色素组织。患者年龄通常在20至50岁之间，没有手术或意外眼外伤史。有色人种更容易受到影响。根据修订的诊断标准，根据眼外表现（神经、听觉和皮肤）的存在，将疾病分类为完全、不完全或可能。VKH的临床病程分为四个阶段：前驱期（类似病毒感染）、葡萄膜炎（双侧弥漫性葡萄膜炎伴乳头炎和渗出性视网膜脱离）、恢复期（组织色素脱失）和慢性复发期（复发性葡萄膜炎和眼部并发症）。VKH的发病机制被认为与针对在黑素细胞上发现的自身抗原的异常T细胞介导的免疫应答有关。VKH与人类白细胞抗原DR 4（HLA-DR 4）和HLA-Dw 53相关，与HLA-DRB 1 *0405单倍型相关风险最强。VKH的诊断是临床的，鉴别诊断包括交感性眼炎、结节病、原发性眼内B细胞淋巴瘤、后巩膜炎和葡萄膜渗出综合征。治疗通常以高剂量口服皮质类固醇开始，但对于无反应的患者或当皮质类固醇副作用不耐受时，可能需要其他免疫调节剂（大多数情况下为环孢菌素）。视力预后一般良好，及时诊断和积极的免疫调节治疗。
Vogt-Koyanagi-Harada disease (VKH) is a multisystem autoimmune disorder principally affecting pigmented tissues in the ocular, auditory, integumentary and central nervous systems. Patients are typically 20 to 50 years old and have no history of either surgical or accidental ocular trauma. Pigmented races are more commonly affected. Depending on revised diagnostic criteria, the disease is classified as complete, incomplete or probable based on the presence of extraocular findings (neurological, auditory and integumentary). The clinical course of VKH is divided into four phases: prodromal (mimics a viral infection), uveitic (bilateral diffuse uveitis with papillitis and exudative retinal detachment), convalescent (tissue depigmentation), and chronic recurrent (recurrent uveitis and ocular complications). The pathogenesis of VKH is thought to be related to an aberrant T cell-mediated immune response directed against self-antigens found on melanocytes. VKH has been linked to human leukocyte antigen DR4 (HLA-DR4) and HLA-Dw53, with strongest associated risk for HLA-DRB1*0405 haplotype. The diagnosis of VKH is clinical, and differential includes sympathetic ophthalmia, sarcoidosis, primary intraocular B-cell lymphoma, posterior scleritis, and uveal effusion syndrome. Treatment is typically initiated with high-dose oral corticosteroids, but other immunomondulatory agents (most oftentimes cyclosporine) may be needed for non-responsive patients or when corticosteroid side-effects are not tolerated. Visual prognosis is generally good with prompt diagnosis and aggressive immunomodulatory treatment.