VKH disease

Vogt-Koyanagi-Harada Disease Vogt-Koyanagi-Harada (VKH) disease is a rare, multisystem autoimmune disorder characterized by chronic, bilateral, diffuse granulomatous uveitis, often associated with neurological, auditory, and integumentary manifestations. It primarily affects individuals with darker skin pigmentation, such as those of Asian, Hispanic, Middle Eastern, and Native American descent.

Clinical Features

• Ocular Manifestations

VKH disease is primarily known for its ocular involvement, which includes:

• Uveitis: Inflammation of the uveal tract, which includes the iris, ciliary body, and choroid.
• Exudative Retinal Detachment: Accumulation of fluid under the retina, leading to detachment.
• Choroiditis: Inflammation of the choroid, the vascular layer of the eye.
• Neurological and Auditory Symptoms
• Meningeal Irritation: Symptoms such as headache, neck stiffness, and photophobia due to inflammation of the meninges.
• Tinnitus and Hearing Loss: Auditory symptoms due to involvement of the inner ear.
• Integumentary Changes
• Vitiligo: Loss of skin pigmentation.
• Alopecia: Hair loss.
• Poliosis: Whitening of hair, including eyelashes and eyebrows.

Pathophysiology

VKH disease is believed to be an autoimmune disorder where the immune system mistakenly attacks melanocyte-associated antigens. This results in inflammation and damage to tissues rich in melanocytes, such as the eyes, skin, and inner ear.

Diagnosis

Diagnosis of VKH disease is primarily clinical, supported by imaging studies and laboratory tests:

• Ophthalmic Examination: To assess the extent of uveitis and retinal detachment.
• Fluorescein Angiography: To visualize retinal and choroidal circulation.
• Lumbar Puncture: To detect pleocytosis in cerebrospinal fluid.

Treatment

The mainstay of treatment for VKH disease is immunosuppression:

• Corticosteroids: High-dose systemic corticosteroids are often used initially.
• Immunosuppressive Agents: Such as azathioprine, cyclosporine, or methotrexate for long-term management.

Prognosis

With prompt and adequate treatment, the prognosis for VKH disease can be favorable, although some patients may experience chronic or recurrent inflammation leading to complications such as cataracts or glaucoma.

Epidemiology

VKH disease is more prevalent in certain ethnic groups, particularly those with darker skin pigmentation. It is rare in Caucasian populations.

See Also

• Uveitis
• Autoimmune Disorders
• Melanocytes

NIH genetic and rare disease info

• NIH info on VKH disease

VKH disease is a rare disease.