Your browser does not support javascript:   Search for gard hereSearch for news-and-events here.

Diseases

Genetic and Rare Diseases Information Center (GARD)

Print friendly version

Vogt-Koyanagi-Harada disease


Other Names for this Disease

  • Uveomenigitic syndrome
  • VKH disease
  • VKH syndrome
  • Vogt-Koyanagi-Harada syndrome
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.

Overview

What is Vogt-Koyanagi-Harada disease?

What are the signs and symptoms of Vogt-Koyanagi-Harada disease?

What causes Vogt-Koyanagi-Harada disease?

How might Vogt-Koyanagi-Harada disease be treated?

What is the long-term outlook for people with Vogt-Koyanagi-Harada disease?

What is Vogt-Koyanagi-Harada disease?

Vogt-Koyanagi-Harada disease (VKH disease) is a condition that involves chronic inflammation of melanocytes, which are specialized cells that produce a pigment called melanin. Melanin is the substance that gives skin, hair, and eyes their color. Melanin is also found in the retina, where it plays a role in normal vision. People with VKH disease usually develop vision and hearing disturbances first, followed by signs of skin problems.[1][2] The most common symptoms include headaches, panuveitis, vitiligo, hair loss (alopecia), and inner ear disturbances. Neurological symptoms may also occur.[3] The exact cause of VKH disease is not well understood, but research suggests it is an autoimmune disease. It is more common in people with darker skin pigmentation including Asian, Middle Eastern, Hispanic, and Native American populations.[1][2]
Last updated: 1/8/2015

What are the signs and symptoms of Vogt-Koyanagi-Harada disease?

Vogt-Koyanagi-Harada (VKH) disease affects the eyes, ears, skin, and nervous system. Early symptoms may include headache, dizziness, neck stiffness, vomiting, and low-grade fever. The symptoms progress to sudden visual loss in one or both eyes; eye pain; eye swelling and irritation (uveitis and choroiditis); and hearing disturbances. Changes in the coloring of the skin and hair are usually seen three months after symptoms first appear, and they are often permanent.[1] Vision and hearing generally improve with medical treatment.[2]
Last updated: 1/8/2015

What causes Vogt-Koyanagi-Harada disease?

The exact cause of Vogt-Koyanagi-Harada (VKH) disease is not well understood, but research suggests it is an autoimmune disease in which the body attacks its own pigment cells (melanocytes), possibly in response to an infection (such as a virus).[2] The strong association between VKH disease and certain ethnic groups suggests that genetic factors may be involved. Genes related to the immune system, namely those genes in the HLA gene family, may play a role in the development of VKH disease.[4]
Last updated: 1/8/2015

How might Vogt-Koyanagi-Harada disease be treated?

Management of Vogt-Koyanagi-Harada (VKH) disease should involve various specialists including dermatologists, ophthalmologists, and neurologists.[3] Experts agree that successful therapy for VKH disease involves early and aggressive treatment with systemic corticosteroids.[5][6][3] For people who don't respond to this treatment or who cannot withstand side effects, immunomodulatory therapy (which alters the body's immune response) should be started.[5] While there is increasing evidence supporting the use of immunomodulatory therapy in almost all affected people, there is ongoing research to determine exactly when this treatment should be started and who should receive it.[5][7] Surgery for glaucoma is necessary in some people.[5] Other treatments for VKH disease are symptomatic and supportive.[6]

You can view more detailed information about the treatment of VKH disease on Medscape's Web site.
Last updated: 1/13/2015

What is the long-term outlook for people with Vogt-Koyanagi-Harada disease?

With early and aggressive treatment, the long-term outlook (prognosis) is usually favorable, although acute hearing and vision changes may occur.[3] The prognosis is improved by the use of early, high-dose corticosteroids during the acute phase of the disease, followed by slowly tapering doses until finished. In most cases, therapy should not be stopped during the 3 months after the onset of the disease. This is because of the high recurrence rate during this period. Many people need a tapering period of at least 3-6 months before the corticosteroids can be stopped.[5]

People who are treated later in the course of the disease have a more guarded prognosis for recovery of visual acuity, and probably have a greater risk for chronic inflammation.[5]

Long-term complications of VKH disease include reversible and irreversible vision loss. Vision loss is often due to cataracts, glaucoma, and choroidal neovascularization (growth of new blood vessels in the choroid layer of the eye). Final visual outcome often depends on the rapidity and appropriateness of treatment. Complications are typically more severe in children than in adults, leading to rapid deterioration in vision.[5]

Neurologic symptoms may persist for weeks, although most signs and symptoms resolve with corticosteroid therapy. Inner ear symptoms typically respond to corticosteroid therapy within weeks to months, and hearing is restored completely in most affected people. Most of the skin symptoms such as hair loss (alopecia), poliosis, and vitiligo persist despite therapy, and some skin changes may be permanent.[5]
Last updated: 1/13/2015

References
  1. Choczaj-Kukula A.. Vogt-Koyanagi-Harada Syndrome. Emedicine. July 15, 2009; http://emedicine.medscape.com/article/1118177-overview. Accessed 10/28/2010.
  2. Fang W, Yang P.. Vogt-Koyanagi-Harada. Current Eye Research. May, 2008; Accessed 10/28/2010.
  3. Olivier Calvetti, Caroline Laurent-Coriat, Michel Paques. VOGT-KOYANAGI-HARADA DISEASE. Orphanet. March, 2009; Accessed 1/12/2015.
  4. Walton RC.. Vogt-Koyanagi-Harada Disease. Emedicine. April 14, 2010; http://emedicine.medscape.com/article/1229432-overview. Accessed 10/28/2010.
  5. R Christopher Walton. Vogt-Koyanagi-Harada Disease. Medscape Reference. February 12, 2014; http://reference.medscape.com/article/1229432-overview. Accessed 1/13/2015.
  6. VOGT-KOYANAGI-HARADA DISEASE. NORD. February 12, 2014; http://www.rarediseases.org/rare-disease-information/rare-diseases/byID/968/viewAbstract. Accessed 1/12/2015.
  7. Urzua CA et. al. Earlier immunomodulatory treatment is associated with better visual outcomes in a subset of patients with Vogt-Koyanagi-Harada disease. Acta Ophthalmol. January 7, 2015; [Epub ahead of print]:


Other Names for this Disease
  • Uveomenigitic syndrome
  • VKH disease
  • VKH syndrome
  • Vogt-Koyanagi-Harada syndrome
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.