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

Marcelo Mendes Lavezzo,
Viviane Mayumi Sakata,
Celso Morita,
Ever Ernesto Caso Rodriguez,
Smairah Frutuoso Abdallah,
Felipe T. G. da Silva,
Carlos Eduardo Hirata and
Joyce Hisae YamamotoEmail author

Orphanet Journal of Rare Diseases201611:29

DOI: 10.1186/s13023-016-0412-4

Received: 17 August 2015

Accepted: 16 March 2016

Published: 24 March 2016

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.