The following information may help to address your question:
What are the signs and symptoms of Vogt-Koyanagi-Harada disease?
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.
Early phase or prodromal symptoms are similar to the flu and may include:
- Low-grade fever
- 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
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.
The chronic stage can last for several months to several years.
During the recurrent stage, people with VKH disease may develop recurrent or chronic eye problems. Common eye complications may include cataracts
, and new, abnormal blood vessels growing under the retina (choroidal neovascularization
), which can lead to scarring (subretinal fibrosis).
Last updated: 10/26/2017
What is the long-term outlook for people with Vogt-Koyanagi-Harada disease?
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
, 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.
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.
Last updated: 10/26/2017
How might Vogt-Koyanagi-Harada disease be treated?
Management of Vogt-Koyanagi-Harada (VKH) disease may involve various specialists including dermatologists
, and neurologists
Experts agree that successful therapy for VKH disease involves early and aggressive treatment with systemic corticosteroids
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.
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.
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.
Surgery for glaucoma is necessary in some people.
Other treatments for VKH disease are symptomatic and supportive.
You can view more detailed information about the treatment of VKH disease on Medscape's Web site
Last updated: 10/26/2017
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