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Vogt-Koyanagi-Harada Disease

.NORD is very grateful to R. Christopher Walton, MD, Director, Uveitis and Ocular Inflammatory Disease Service and Baptist Memorial Health Care Professor of Ophthalmology at the University of Tennessee College of Medicine, for assistance in the preparation of this report.

Synonyms of Vogt-Koyanagi-Harada Disease

  • alopecia-poliosis-uveitis-vitiligo-deafness-cutaneous-uveo-oto syndrome
  • Harada syndrome
  • uveomeningitis syndrome
  • VKH syndrome

Disorder Subdivisions

  • No subdivisions found.

General Discussion

Vogt-Koyanagi-Harada disease is a rare disorder of unknown origin that affects many body systems, including as the eyes, ears, skin, and the covering of the brain and spinal cord (the meninges). The most noticeable symptom is a rapid loss of vision. There may also be neurological signs such as severe headache, vertigo, nausea, and drowsiness. Loss of hearing, and loss of hair (alopecia) and skin color may occur along, with whitening (loss of pigmentation) of the hair and eyelashes (poliosis).

Symptoms

Vogt-Koyanagi-Harada disease is initially characterized by headaches, very deep pain in the eyes, dizziness (vertigo), and nausea. These symptoms are usually followed in a few weeks by eye inflammation (uveitis) and blurring of vision. This may occur in both eyes at the same time or in one eye first and, a few days later, in the other. The retina may detach and hearing loss may become apparent.

The chronic 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), as well as the development of small yellow nodules in parts of the retina. Skin changes may include the development of smooth, white patches in the skin caused by the loss of pigment-producing cells (vitiligo). These white patches are usually distributed over the head, eyelids and torso. The chronic stage can last for several months to several years.

In many individuals, treatment improves sight and hearing. However, there may be some permanent problems, including vision and hearing deficits, and hair loss with associated loss of color of the hair, eyelashes, and skin. Lasting visual effects may include the development of secondary glaucoma and cataracts.

Causes

The exact cause of Vogt-Koyanagi-Harada disease is unknown. It is thought by researchers to be an immune response to the human leukocyte antigen (HLA). These are genetic markers located on chromosome 6 that react specifically with a particular antibody. This genetic defect may predispose persons who carry this antigen to develop Vogt-Koyanagi-Harada disease.

Autoimmune disorders are caused when the body's natural defenses (antibodies, lymphocytes, etc.) against invading organisms suddenly begin to attack perfectly healthy tissue.

Some researchers also think that there may be a genetic predisposition for the disease since it has occurred in a brother and sister and in a set of twins. A genetic predisposition means that a person may carry a gene for a disease but it may not be expressed unless something in the environment triggers the disease.

Affected Populations

Vogt-Koyanagi-Harada disease is a rare disease that affects males and females in equal numbers. The disorder is more prevalent in Oriental, Hispanic, and American Indian populations than in people who trace their ancestry to northern Europe. Onset typically occurs at around 30 or 40 years of age, but cases have been reported among children as young as four years old.

Related Disorders

Symptoms of the following disorders can be similar to those of Vogt-Koyanagi-Harada disease or may occur in conjunction with Vogt-Koyanagi-Harada disease.

Alopecia is unpredictable hair loss due to an unknown cause. Regrowth of hair may or may not occur. Hair loss is usually confined to the head and face, although the entire body may be involved. (For more information on this disorder, choose "Alopecia" as your search term in the Rare Disease Database.)

Vitiligo is characterized by spots on the skin with decreased pigmentation. These areas are usually sharply demarcated with increased coloring (hyperpigmentation) on the borders, and are often symmetrical in shape. The face, neck, hands, abdomen, and thighs are most often affected. The hair and skin in the area are usually white. The white areas are prone to sunburn and should be protected from sunlight. (For more information on this disorder, choose "Vitiligo" as your search term in the Rare Disease Database.)

Glaucoma may occur as a secondary characteristic of VKH disease. It is characterized by increased pressure within the eye. If left untreated the increased pressure may affect the lens and optic nerve of the eye, resulting in eventual blindness. Some symptoms for people to be aware of are: blurred vision, rainbow colored halos around lights, and loss of side vision resulting in "tunnel vision."

Standard Therapies

Diagnosis
The diagnostic criteria for VKH disease includes inflammation of both eyes, no evidence of another ocular disease causing the inflammation, and no history of trauma or ocular surgery. An international group of experts has established three categories of disease:

a. Complete VKH disease: diffuse choroiditis involving both eyes that may include serous retinal detachments. Inflammation of the iris and ciliary body may develop in some patients. The complete form of the disease includes neurologic signs such as ringing in the ears (tinnitus), neck stiffness, and/or cells in the cerebrospinal fluid (pleocytosis) as well as dermatological signs such as white patches on the arms or torso, sudden loss of hair (alopecia), or loss of color of the hair, eyelashes or eyelids (poliosis)

b. Incomplete VKH disease: similar eye disease as patients with complete VKH disease but do not have both neurologic and dermatological signs. Patients must have either the neurologic manifestations or dermatological signs.

c. Probable VKH disease: similar eye disease as patients with complete VKH disease but without neurologic and dermatological signs.

Treatment
Standard treatment of Vogt-Koyanagi-Harada disease is the use of high-dose systemic steroid drugs initially and often followed by immunosuppressive therapy. Testing used by an ophthalmologist or neurologist to determine if this disease is present include a spinal tap, x-ray of the blood vessels after the injection of dye (angiography), and ultrasound. Other treatment is symptomatic and supportive.

Investigational Therapies

Information on current clinical trials is posted on the Internet at www.clinicaltrials.gov. All studies receiving U.S. government funding, and some supported by private industry, are posted on this government web site.

For information about clinical trials being conducted at the NIH Clinical Center in Bethesda, MD, contact the NIH Patient Recruitment Office:

Tollfree: (800) 411-1222
TTY: (866) 411-1010
Email: prpl@cc.nih.gov

For information about clinical trials sponsored by private sources, contact:
www.centerwatch.com

For information about clinical trials conducted in Europe, contact:
https://www.clinicaltrialsregister.eu/

Intravenous immunoglobulin (IVIg) therapy is being studied as a potential treatment option for individuals with Vogt-Koyanagi-Harada disease. Initial findings demonstrate that IVIg therapy was effective in improving symptoms associated with the disorder. More research is necessary to determine the long-term safety and effectiveness of this potential treatment for individuals with Vogt-Koyanagi-Harada disease.

Contact for additional information about Vogt-Koyanagi-Harada disease:

R. Christopher Walton, MD
Director, Uveitis and Ocular Inflammatory Disease Service
Baptist Memorial Health Care Professor of Ophthalmology
University of Tennessee College of Medicine

Vogt-Koyanagi-Harada Disease Resources

NORD Member Organizations:

(To become a member of NORD, an organization must meet established criteria and be approved by the NORD Board of Directors. If you're interested in becoming a member, please contact Susan Olivo, Membership Manager, at solivo@rarediseases.org.)

Other Organizations:

References

TEXTBOOKS
Foster RE. Vogt-Kayonagi-Harada Syndrome. In: NORD Guide to Rare Disorders. Lippincott Williams & Wilkins. Philadelphia, PA. 2003:36-37.

Rowland LP., ed. Merritt's Neurology. 10th ed. Lippincott Williams & Wilkins. Philadelphia, PA. 2000:122-23;126-27.

Adams RD, Victor M, Ropper AA., eds. Principles of Neurology. 6th ed. McGraw-Hill Companies. New York, NY; 1997:748.

Kanski JJ., ed. Clinical Ophthalmology. 4th ed. Butterworth-Heinemann. Oxford, UK; 1999:282-83.

Newell FW., ed. Ophthalmology: Principles and Concepts. 7th ed. Mosby Year Book, St. Louis, MO; 1991:335.

REVIEW ARTICLES
Damico FM, Kiss S, Young LH. Vogy-Koyanagi-Harada disease. Semin Ophthalmol. 2005;20:183-90.

Stanga PE, Lim JI, Hamilton P. Indocyanine green angiography in chorioretinal diseases: indications and interpretation: an evidence-based update. Ophthalmology. 2003;110:15-21.

Read RW. Vogt-Koyanagi-Harada disease. Ophthalmol Clin North Am. 2002;15:333-41, vii.

Boyd SR, Young S, Lightm,an S. Immunopathology of the noninfectious posterior and intermediate uveitides. Surv Ophthalmol. 2001;46:209-33.

JOURNAL ARTICLES
Bykhovskaya I, Thorne JE, Kempen JH, Dunn JP, Jabs DA. Vogy-Koyanagi disease: clinical outcomes. Am J Ophthalmol. 2005;140:674-8.

Gonzalez-Delgado M, Gonzalez C, Blazquez JI, et al., Intravenous immunoglobulin therapy in Vogt-Koyanagi-Harada syndrome. Neurologia. 2004;19:401-3.

Ganesh SK, Padmaja Babu K, Biswas J. Cataract surgery inpatients with Vogt-Koyanagi-Harada syndrome. J Cataract Refract Surg. 2004;30:95-100.

Sheu SJ, Kou HK, Chen JF. Prognostic factors for Vogt-Koyanagi-Harada disease. J China Med Assoc. 2003;66:148-54.

Kouda N, Sasaki H, Harada S, et al. Early manifestation of vogt-koyanagi-harada disease as unilateral posterior scleritis. Jpn J Ophthalmol. 2002;46:590-93.

Schalenbourg A, Leys A, De Courten C, et al. Corticosteroid-induced central serous chorioretinopathy inpatients with ocular inflammatory disorders. Klin Monatsbla Augenheilkd. 2002;219:264-67.

Imai Y, Sugita M, Nakamura S, et al. Cytokine production and helper T-cell subsets in Vogt-Koyanagi-Harada disease. Cur Eye Res. 2001;22:312-18.

Kamondi A, Szegedi A, Papp A, et al. Vogt-Koyanagi-Harada disease presenting initially as aseptic meningoencephalitis. Eur J Neurol. 2000;7:719-22.

INTERNET
Walton RC. Vogt-Koyanagi-Harada Disease. Medscape. Updated: Oct 31, 2012. http://emedicine.medscape.com/article/1229432-overview Accessed Feb 6, 2014.

The information in NORD’s Rare Disease Database is for educational purposes only. It should never be used for diagnostic or treatment purposes. If you have questions regarding a medical condition, always seek the advice of your physician or other qualified health professional. NORD’s reports provide a brief overview of rare diseases. For more specific information, we encourage you to contact your personal physician or the agencies listed as “Resources” on this report.

Report last updated: 2014/02/12 00:00:00 GMT+0

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