Vogt-Koyanagi-Harada disease

Surv Ophthalmol. 2017 Jan-Feb;62(1):1-25. doi: 10.1016/j.survophthal.2016.05.002. Epub 2016 May 27.


Vogt-Koyanagi-Harada disease, a severe bilateral granulomatous intraocular inflammation associated with serous retinal detachments, disk edema, and vitritis, with eventual development of a sunset glow fundus, is an autoimmune inflammatory condition mediated by T cells that target melanocytes in individuals susceptible to the disease. Vogt-Koyanagi-Harada disease presents clinically in 4 different phases: prodromal, uveitic, convalescent, and recurrent, with extraocular manifestations including headache, meningismus, hearing loss, poliosis, and vitiligo, to varying degrees. There have been considerable advances in imaging modalities resulting in earlier diagnosis and improved understanding of this disease. Ocular coherence tomography has replaced other imaging modalities in the diagnosis of acute and chronic Vogt-Koyanagi-Harada disease by revealing exudative detachments of the retina in the acute stage, along with choroidal thickening and demonstrating choroidal thinning in the chronic stage. Treatment of this disease is initially with corticosteroids, with a transition to immunomodulatory drugs for long-term control. Patients with Vogt-Koyanagi-Harada disease can have good final outcomes if treated promptly and aggressively and thus avoid complications such as sunset glow fundus, cataracts, glaucoma, subretinal fibrosis, and choroidal neovascularization.

Keywords: Vogt-Koyanagi-Harada; autoimmunity; fluorescein angiography; fundus autofluorescence; immune disorders; immunomodulators; inflammation; optical coherence tomography; uveitis.

Publication types

  • Review

MeSH terms

  • Autoimmunity*
  • Diagnostic Techniques, Ophthalmological*
  • Humans
  • Immunologic Factors / therapeutic use*
  • Uveomeningoencephalitic Syndrome / diagnosis
  • Uveomeningoencephalitic Syndrome / drug therapy*
  • Uveomeningoencephalitic Syndrome / immunology
  • Visual Acuity*


  • Immunologic Factors