Vogt-Koyanagi-Harada disease

Información en español Title

Other Names:

VKH syndrome; Vogt-Koyanagi-Harada syndrome; Uveomenigitic syndrome; VKH syndrome; Vogt-Koyanagi-Harada syndrome; Uveomenigitic syndrome; VKH disease See More

Categories:

Eye diseases; Nervous System Diseases

Summary Summary

Vogt-Koyanagi-Harada disease (VKH disease) is a disease that affects several parts of the body, including the eyes, ears, nervous system, and skin. The signs and symptoms of VKH disease are caused by chronic inflammation of melanocytes. Melanocytes 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 other parts of the body, such as the retina of the eyes, where it plays a role in normal vision, and in the inner ear, where it is thought to be involved in hearing.^[1]^[2]^[3] People with VKH disease usually develop vision and hearing problems first, followed by signs of skin problems.^[1]^[2] The most common symptoms include headaches, inflammation of all layers of the colored part of the eye (panuveitis), white patches of skin (vitiligo), hair loss (alopecia), dizziness and nausea (inner ear related problems), and vision and hearing loss. Neurological symptoms may also occur.^[4] 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 color including Asian, Middle Eastern, Hispanic, and Native American populations.^[1]^[2] The disease is treated with corticosteroids and other medications.^[1]

Last updated: 10/26/2017

Symptoms Symptoms

The first symptoms of Vogt-Koyanagi-Harada (VKH) disease are usually eye problems, such as sudden loss of vision, eye pain, and sensitivity to light. Sometimes there are also other symptoms as well, including hearing problems and dizziness. After weeks or months, most people also have skin problems. According to the progression of the symptoms there are 4 stages of the disease: early phase or prodromal, acute uveitic stage, convalescent stage, and chronic recurrent stage.^[1]^[5]

Early phase or prodromal symptoms are similar to the flu and may include:^[1]^[5]

Headache
Low-grade fever
Dizziness
Neck stiffness and pain on the back of the head (meningismus)
Nausea and vomiting
Hearing loss and inner ear related disorders, including distorted hearing (dysacusis), ringing in the ears (tinnitus), and a kind of dizzy, spinning sensation (vertigo)

There may be other less common symptoms such as double vision and drooping of the eyelids due to cranial nerve palsies.^[1]

These symptoms are usually followed in a few weeks by eye inflammation (panuveitis) that may occur in both eyes at the same time or in one eye first and, a few days later, in the other. The symptoms may include:

Sudden vision loss in one or both eyes
Eye pain
Eye swelling and irritation
Dark, floating spots in the vision (floaters) indicating retinal detachment

The convalescent stage follows in a few weeks. This stage is characterized by changes in the eyes and skin. The changes in the eyes may include loss of color in the layer of the eye filled with blood vessels that nourish the retina (choroid) resulting in an orange-red discoloration or "sunset glow fundus" (due to the resolution of the retinal detachments), as well as the development of small yellow nodules in parts of the retina. Changes in the coloring of the skin and hair are usually seen three months after symptoms first appear, and they are often permanent.^[1]^[2] The chronic stage can last for several months to several years.^[1]

During the recurrent stage, people with VKH disease may develop recurrent or chronic eye problems. Common eye complications may include cataracts, glaucoma, and new, abnormal blood vessels growing under the retina (choroidal neovascularization), which can lead to scarring (subretinal fibrosis).^[1]

Last updated: 10/26/2017

This table lists symptoms that people with this disease may have. For most diseases, symptoms will vary from person to person. People with the same disease may not have all the symptoms listed. This information comes from a database called the Human Phenotype Ontology (HPO) . The HPO collects information on symptoms that have been described in medical resources. The HPO is updated regularly. Use the HPO ID to access more in-depth information about a symptom.

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Medical Terms	Other Names	Learn More: HPO ID
80%-99% of people have these symptoms
Abnormal eyebrow morphology	Abnormality of the eyebrow	0000534
Abnormal eyelash morphology	Abnormal eyelashes Abnormality of the eyelashes Eyelash abnormality [ more ]	0000499
Cognitive impairment	Abnormality of cognition Cognitive abnormality Cognitive defects Cognitive deficits Intellectual impairment Mental impairment [ more ]	0100543
Hypopigmented skin patches	Patchy loss of skin color	0001053
Poliosis	Patch of white hair White patch [ more ]	0002290
Premature graying of hair	Early graying Premature graying Premature greying Premature hair graying [ more ]	0002216
Sensorineural hearing impairment		0000407
Sparse scalp hair	Scalp hair, thinning Sparse, thin scalp hair sparse-absent scalp hair [ more ]	0002209
Vitiligo	Blotchy loss of skin color	0001045
30%-79% of people have these symptoms
Cataract	Clouding of the lens of the eye Cloudy lens [ more ]	0000518
Glaucoma		0000501
Retinal detachment	Detached retina	0000541
Short stature	Decreased body height Small stature [ more ]	0004322
Visual impairment	Impaired vision Loss of eyesight Poor vision [ more ]	0000505

Showing of 14 |

Do you have more information about symptoms of this disease? We want to hear from you.

Last updated: 12/1/2018

Do you have updated information on this disease? We want to hear from you.

Cause Cause

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.^[1]

Last updated: 10/26/2017

Treatment Treatment

Management of Vogt-Koyanagi-Harada (VKH) disease may involve various specialists including dermatologists, ophthalmologists, and neurologists.^[4] Experts agree that successful therapy for VKH disease involves early and aggressive treatment with systemic corticosteroids (steroids).^[1]^[4]^[5] Even when the symptoms of VKH disease get better with steroid therapy, many people need to slowly decrease the dose of steroid over a period of 3-6 months so that their symptoms do not return.^[1]

For people who don't respond to steroids or who cannot tolerate the side effects, a type of therapy that changes the response of the body's immune system called immunomodulatory therapy may be tried.^[1] While there is increasing evidence supporting the use of immunomodulatory therapy in almost all people with VKH disease, there is ongoing research to determine exactly when this treatment should be started and who should receive it.^[1]^[6] Surgery for glaucoma is necessary in some people.^[1] Other treatments for VKH disease are symptomatic and supportive.^[5]

You can view more detailed information about the treatment of VKH disease on Medscape's Web site.

Last updated: 10/26/2017

Prognosis Prognosis

Treatment of Vogt-Koyanagi-Harada (VHK) disease usually improves both the vision and hearing loss caused by the disease. People who are treated early in the course of the disease tend to recover more fully than those who are treated later. Other factors that may affect recovery include response to treatment and severity of symptoms. Full recovery of vision is also dependent on the ability to treat secondary visual complications including cataracts, glaucoma, and/or the growth of new blood vessels in the choroid layer of the eye (choroidal neovascularization). Complications are typically more severe in children than in adults, which can lead to greater risk of permanent vision loss for children.^[1]

Even with treatment early in the course of VKH disease, it can take a few weeks or months for neurological symptoms (ringing in the ears and stiff neck) and inner ear symptoms (dizziness and nausea) to get better completely. Some people do not fully recover all of their sight or hearing. Hair loss (alopecia), hair color changes, and/or the white patches of skin (vitiligo) may be permanent as well.^[1]

Last updated: 10/26/2017

Research Research

Research helps us better understand diseases and can lead to advances in diagnosis and treatment. This section provides resources to help you learn about medical research and ways to get involved.

Clinical Research Resources

ClinicalTrials.gov lists trials that are related to Vogt-Koyanagi-Harada disease. Click on the link to go to ClinicalTrials.gov to read descriptions of these studies.

Please note: Studies listed on the ClinicalTrials.gov website are listed for informational purposes only; being listed does not reflect an endorsement by GARD or the NIH. We strongly recommend that you talk with a trusted healthcare provider before choosing to participate in any clinical study.
Orphanet lists European clinical trials, research studies, and patient registries enrolling people with this condition.

Organizations Organizations

Support and advocacy groups can help you connect with other patients and families, and they can provide valuable services. Many develop patient-centered information and are the driving force behind research for better treatments and possible cures. They can direct you to research, resources, and services. Many organizations also have experts who serve as medical advisors or provide lists of doctors/clinics. Visit the group’s website or contact them to learn about the services they offer. Inclusion on this list is not an endorsement by GARD.

Organizations Supporting this Disease

American Uveitis Society
PO Box 016880
Miami, FL 33101-6880
Telephone: 305-326-6377
Fax: 305-326-6071
Website: http://www.uveitissociety.org/

Organizations Providing General Support

American Autoimmune Related Diseases Association (AARDA)
22100 Gratiot Avenue
Eastpointe, MI 48021
Toll-free: 800-598-4668
Telephone: 586-776-3900
Fax: 586-776-3903
E-mail: aarda@aarda.org
Website: https://www.aarda.org/

Do you know of an organization? We want to hear from you.

Learn More Learn More

These resources provide more information about this condition or associated symptoms. The in-depth resources contain medical and scientific language that may be hard to understand. You may want to review these resources with a medical professional.

Where to Start

The National Organization for Rare Disorders (NORD) has a report for patients and families about this condition. NORD is a patient advocacy organization for individuals with rare diseases and the organizations that serve them.

In-Depth Information

Medscape Reference provides information on this topic. You may need to register to view the medical textbook, but registration is free.
The Monarch Initiative brings together data about this condition from humans and other species to help physicians and biomedical researchers. Monarch’s tools are designed to make it easier to compare the signs and symptoms (phenotypes) of different diseases and discover common features. This initiative is a collaboration between several academic institutions across the world and is funded by the National Institutes of Health. Visit the website to explore the biology of this condition.
Orphanet is a European reference portal for information on rare diseases and orphan drugs. Access to this database is free of charge.
PubMed is a searchable database of medical literature and lists journal articles that discuss Vogt-Koyanagi-Harada disease. Click on the link to view a sample search on this topic.

GARD Answers GARD Answers

Questions sent to GARD may be posted here if the information could be helpful to others. We remove all identifying information when posting a question to protect your privacy. If you do not want your question posted, please let us know. Submit a new question

My daughter was diagnosed 10 years ago. Does this syndrome progressively worsen? Can it be controlled by medication and a healthy lifestyle? See answer
What is the cause of Vogt-Koyanagi-Harada syndrome? See answer

Have a question? Contact a GARD Information Specialist.

References References

Walton RC. Vogt-Koyanagi-Harada Disease. Medscape Reference. 2016; http://reference.medscape.com/article/1229432-overview.
Fang W & Yang P. Vogt-Koyanagi-Harada. Current Eye Research. Jul 2008; 33(7):517-23. https://www.ncbi.nlm.nih.gov/pubmed/18600484.
Bismuth K & Arnheiter H. Neural Crest Cell Diversification and Specification: Melanocytes. Encyclopedia of Neuroscience. 2009; 143-151. http://www.sciencedirect.com/science/article/pii/B978008045046901038X.
Calvetti O, Laurent-Coriat C, Paques M. Vogt-Koyanagi-Harada Disease. Orphanet. March 2009; http://www.orpha.net/consor/cgi-bin/OC_Exp.php?lng=en&Expert=3437.
Walton RC. Vogt-Koyanagi-Harada Disease. National Organization of Rare Disorders (NORD). February 12, 2014; https://rarediseases.org/rare-diseases/vogt-koyanagi-harada-disease/.
Urzua CA, Velasquez V, Sabat P, Berger O, Ramirez S, Goecke A, Vásquez DH, Gatica H, Guerrero J.. Earlier immunomodulatory treatment is associated with better visual outcomes in a subset of patients with Vogt-Koyanagi-Harada disease. Acta Ophthalmol. Sept 2015; 93(6):e475-80. https://www.ncbi.nlm.nih.gov/pubmed/25565265.