Vogt-Koyanagi-Harada disease

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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) affects the eyes, ears, nervous system, and skin. The symptoms of VKH occur in different phases. The early symptoms include headaches, eye pain, and dizziness. These are followed by hearing loss and inflammation of the eye (panuveitis). Later symptoms include development of white patches of skin and hair (vitiligo), hair loss (alopecia), cataracts, and glaucoma. The exact cause of VKH disease is unknown, but the symptoms are thought to be due to an abnormal response of the immune system to a viral infection. Genetic factors may be involved. Diagnosis of VKH disease is based on the symptoms, clinical exam, and imaging studies. Other more common diseases may need to excluded before a diagnosis of VKH disease is made. VKH disease is treated with corticosteroids and other medications.^[1]^[2]^[3]^[4]

Last updated: 8/24/2020

Symptoms Symptoms

The following list includes the most common signs and symptoms in people with Vogt-Koyanagi-Harada disease. These features may be different from person to person. Some people may have more symptoms than others and symptoms can range from mild to severe. This list does not include every symptom or feature that has been described in this condition.^[2]^[3]

The symptoms of Vogt-Koyanagi-Harada(VKH) disease occur in different phases. In the early phase, symptoms may include:

Headache
Dizziness (vertigo)
Neck stiffness
Nausea and vomiting
Hearing loss
Ringing in the ears (tinnitus)

The uveitis phase occurs a few weeks after the early phase. In this phase, symptoms may include:

Blurry vision in one or both eyes
Inflammation of the eyes (panuveitis)
Floating spots in the vision (floaters) that are signs of retinal detachment

The convalescent phase usually occurs a few weeks to months after the uveitis phase. In this phase, symptoms may include:

Patches of white skin (vitiligo)
Patches of white hair, eyelashes, and eyebrows (poliosis)
Hair loss (alopecia)

The recurrent phase occurs in about half of the people with VKH disease. Symptoms may include:

Clouding of the lens (cataracts)
Build-up of pressure in the eyes (glaucoma)
Abnormal blood vessels growth under the retina (choroidal neovascularization)

Most people with VKH disease develop symptoms in both eyes. Vision and hearing problems can improve with treatment. The skin changes and hair loss may be permanent.

Last updated: 8/24/2020

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.

Showing of 14 |

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	Reduced/lack of hair on scalp 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 |

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Last updated: 1/1/2021

Cause Cause

The exact cause of Vogt-Koyanagi-Harada (VKH) disease is not known. It is thought to be due to an abnormal immune response in which the body attacks its own pigment cells (melanocytes). In addition, genetic factors may play a role.^[3]^[4]

Last updated: 8/25/2020

Diagnosis Diagnosis

Vogt-Koyanagi-Harada (VKH) disease is diagnosed based on the symptoms, clinical exam, eye exam, and imaging studies. In addition, other more common conditions may need to be excluded before VKH disease can be diagnosed.^[3] A set of standard signs and symptoms have been published to help guide the diagnosis of VKH disease.^[5]

Last updated: 8/25/2020

Treatment Treatment

Treatment of Vogt-Koyanagi-Harada (VKH) disease is focused on managing the symptoms. Treatment usually involves early and aggressive treatment with systemic corticosteroids (steroids). Other medications may be used as well.^[2]^[3]

Specialists involved in the care of someone with VKH disease may include:^[6]

Dermatologist
Ophthalmologist
Neurologist

Last updated: 8/25/2020

Statistics Statistics

In the United States, the incidence of Vogt-Koyanagi-Harada (VKH) disease is thought to be about 1.5 to 6 per 1 million patients. The incidence may be higher in other countries. People of Asian, Middle Eastern, Hispanic and Native American descent have a higher incidence of VKH disease.^[4]

Last updated: 8/25/2020

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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
Office of the Executive Secretary
700 18th Street South Suite 601
Birmingham, AL 35233
Telephone: +1-205-325-8507
Website: https://uveitissociety.org/

Organizations Providing General Support

American Autoimmune Related Diseases Association (AARDA)
19176 Hall Road, Suite 130
Clinton Township, MI 48038
Toll-free: 800-598-4668
Telephone: 586-776-3900
Fax: 586-776-3903
E-mail: aarda@aarda.org
Website: https://www.aarda.org/

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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.

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References References

O'Keefe GA, Rao NA. Vogt-Koyanagi-Harada disease. Surv Ophthalmol. 2017; 62(1):1-25. https://pubmed.ncbi.nlm.nih.gov/27241814.
Baltmr A, Ligtman S, Tomkins-Netzer O. Vogt-Koyanagi-Harada syndrome - current perspectives. Clin Ophthalmol. 2016; 10:2345-2361. https://pubmed.ncbi.nlm.nih.gov/27932857.
Street D, Sivaguru A, Sreekantam S, Mollan SP. Vogt-Koyanagi-Harada disease. Pract Neurol. 2019; 19(4):364-367. https://pubmed.ncbi.nlm.nih.gov/30890584.
Lavezzo MM, Sakata VM, Morita C, et al. Vogt-Koyanagi-Harada disease: review of a rare autoimmune disease targeting antigens of melanocytes. Orphanet J Rare Dis. 2016. 2016; 11:29. https://pubmed.ncbi.nlm.nih.gov/27008848.
Yang P, Zhong Y, Du L, et al. Development and Evaluation of Diagnostic Criteria for Vogt-Koyanagi-Harada Disease. JAMA Ophthalmol. 2018; 136(9):1025-1031. https://pubmed.ncbi.nlm.nih.gov/29978190.
Walton RC. Vogt-Koyanagi-Harada Disease. National Organization of Rare Disorders (NORD). February 12, 2014; https://rarediseases.org/rare-diseases/vogt-koyanagi-harada-disease/.