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Vogt-Koyanagi-Harada Syndrome

• Definition of Vogt-Koyanagi-Harada Syndrome
• Causes and Risk Factors of Vogt-Koyanagi-Harada Syndrome
• Symptoms of Vogt-Koyanagi-Harada Syndrome
• Diagnosis of Vogt-Koyanagi-Harada Syndrome
• Treatment of Vogt-Koyanagi-Harada Syndrome
• What Questions To Ask Your Doctor About Vogt-Koyanagi-Harada Syndrome

Definition of Vogt-Koyanagi-Harada Syndrome

Vogt-Koyanagi-Harada syndrome is a rare disease of the eyes characterized by bilateral uveitis (inflammation of the uveal tract consisting of the iris, ciliary body, and choroid), alopecia (hair loss), poliosis (depigmented eyelashes, eyebrows, or hair), vitiligo (a skin disease with depigmentation), and hearing loss.

Causes and Risk Factors of Vogt-Koyanagi-Harada Syndrome

The exact cause of Vogt-Koyanagi-Harada syndrome is not known, but an immune response to a human leukocyte antigen (HLA) is suspected. Some researchers have found a genetic predisposition for the disease since it has occurred in a brother and sister and in a set of twins.

The syndrome affects males and females equally. The disorder is more prevalent among Asians and Native Americans.

Symptoms of Vogt-Koyanagi-Harada Syndrome

The most noticeable symptom is a rapid loss of vision. Initial onset of Vogt-Koyanagi-Harada syndrome is characterized by severe headache, deep pain in the eye, vertigo (feeling that the room is spinning), and nausea, followed in a few weeks by uveitis and vision loss.

The other eye may become affected about 2 weeks later. The retina may detach, and hearing loss may become apparent. Facial nerve palsies (weakness) and rigidity as well as gait (walking) disturbance can occur.

Diagnosis of Vogt-Koyanagi-Harada Syndrome

Diagnosis is made by medical history and physical examination including an eye exam.

Treatment of Vogt-Koyanagi-Harada Syndrome

High dose systemic steroid drug therapy is often the treatment of choice. Other treatment is symptomatic and supportive.

After treatment, sight and hearing usually return. However, there may be some permanent hair loss with associated depigmentation of the hair, eyelashes, and skin. Lasting visual effects include the development of secondary glaucoma and cataracts.

What Questions To Ask Your Doctor About Vogt-Koyanagi-Harada Syndrome

What is the probable cause of the condition?

How are the eyes affected?

Will there be permanent vision loss?

What treatments are available?

Will you prescribe steroid drugs?

Will other eye problems and hair loss develop?

What is the prognosis?