Vogt-Koyanagi-Harada disease: review of a rare autoimmune disease targeting antigens of melanocytes

Abstract

Vogt-Koyanagi-Harada disease (VKHD) is a rare granulomatous inflammatory disease that affects pigmented structures, such as eye, inner ear, meninges, skin and hair. This disease is mainly a Th1 lymphocyte mediated aggression to melanocytes after a viral trigger in the presence of HLA-DRB1*0405 allele. The absence of ocular trauma or previous intraocular surgery sets VKHD appart from sympathetic ophthalmia, its main differential diagnosis. The disease has an acute onset of bilateral blurred vision with hyperemia preceded by flu-like symptoms. The acute uveitic stage is characterized by a diffuse choroiditis with serous retinal detachment and optic disc hyperemia and edema. Fluorescein angiography in this phase demonstrates multiple early hyperfluorescent points. After the acute uveitic stage, ocular and integumentary system pigmentary changes may appear. Ocular findings may be accompanied by lymphocytic meningitis, hearing impairment and/or tinnitus in a variable proportion of patients. Prompt diagnosis followed by early, aggressive and long-term treatment with high-dose corticosteroids is most often ensued by good visual outcomes. However, some patients may experience chronic uveal inflammation with functional eye deterioration. The current review discusses the general features of VKHD, including epidemiology, classification into categories, differential diagnosis and current therapeutic approaches.

Differentiating Vogt-Koyanagi-Harada syndrome from recurrent optic neuritis: a case report and review of the literature concerning Hispanic patients

“…VKH is a rare disease in Europe. In Switzerland, where the first patient was described by Vogt [5], VKH represents 0.7% of all uveitis [15]. Two studies from the North of Italy, where our hospital is located, report VKH as accounting for 1,37-4,1% of all uveitis (the former being a percentage even lower then the rest of Europe) [16,17].…”

“…It predominantly affects Asian people, but also people with darker skin pigmentation such as Native Americans and Hispanics (Mestizos), whose ancestors moved from Asia across the Bering strait to North America and further down to Central and South America [3,4]. Genetic susceptibility is linked to certain HLA alleles, the most relevant being HLA-DRB1*0405, which has been reported as the predominant allele across different ethnic groups [5]. The typical age of incidence is between 20 and 50 years, and women are affected twice than men.…”

Association of Long Noncoding RNAs Polymorphisms With Ankylosing Spondylitis, Vogt-Koyanagi-Harada Disease, and Behcet's Disease

“…BD is an inflammatory disorder with a complex genetic background characterized by uveitis, oral aphthae, skin lesions, and genital ulcers, whereas VKH disease is a systemic autoimmune disease that targets melanocytes not only in the uvea, but also elsewhere in the body, resulting in multiple systemic abnormalities leading to dysacusis, alopecia, poliosis, and vitiligo. 62,63 As mentioned above, rs4937362 was associated with AS in our Chinese Han population but not with the uveitis entities tested. The SNP rs4937362 is located at 11q24.3 in lncRNA RP11-264E20.1 and has been shown to be strongly associated with follicular lymphoma (P ¼ 6.76 3 10 À11 ).…”

Progressive Depigmentation in a Patient with Panuveitis and Meningitis

“…However, individuals of African descent are uncommonly affected [2]. Among all cases of uveitis, VKH accounts for approximately 1-4% of cases in the US [1,2]. …”

“…The disease is exceptionally rare with an incidence of approximately 1.5-6 per 1 million patients in the US [1]. …”