Vogt-Koyanagi-Harada disease

Vogt-Koyanagi-Harada disease (often called VKH disease) is a rare condition in which the body's immune system mistakenly attacks cells that contain melanin, a pigment found in the eyes, skin, inner ear, and the membranes surrounding the brain. It is not inherited in a simple genetic pattern but is considered an autoimmune disorder that occurs in people with certain genetic predispositions, particularly specific HLA (human leukocyte antigen) types. The disease typically progresses through several stages. It often begins with headaches, neck stiffness, fever, and flu-like symptoms. Within days, patients develop serious eye inflammation, including blurred vision, eye pain, sensitivity to light, and sometimes detachment of the retina. If untreated, VKH can lead to permanent vision loss. Later stages may involve whitening of the hair (poliosis), loss of skin color in patches (vitiligo), and hearing problems such as ringing in the ears or hearing loss. Treatment focuses on suppressing the immune system to control inflammation. High-dose corticosteroids are the first-line therapy, often followed by steroid-sparing immunosuppressive medications for long-term management. Early and aggressive treatment is critical to preserving vision and preventing recurrences. With proper treatment, many patients can maintain useful vision, though chronic or recurrent inflammation may lead to complications like glaucoma, cataracts, or ongoing vision problems.

Data sourced from FDA regulatory filings and ClinicalTrials.gov. Updated periodically.

Bring these to your next appointment

• Q1.What stage is my VKH disease, and how much damage has already occurred in my eyes?,How long will I need to take immunosuppressive medications, and what are the risks?,What signs should I watch for that would indicate a disease flare?,Are there newer or biologic treatments that might be appropriate for me?,How often will I need eye exams and blood tests to monitor my condition?,What is my long-term outlook for preserving my vision?,Should I see other specialists such as a dermatologist or audiologist for my other symptoms?

Common questions about Vogt-Koyanagi-Harada disease

Related conditions

Other rare diseases commonly researched alongside Vogt-Koyanagi-Harada disease.

• Behçet disease→
• Birdshot chorioretinopathy→
• Sympathetic ophthalmia→