Vogt–Koyanagi–Harada syndrome

Vogt–Koyanagi–Harada syndrome (VKH) is a rare autoimmune disease that affects multiple systems in the body, including the eyes, ears, skin, and the central nervous system. The syndrome is characterized by bilateral uveitis, which can lead to complications such as glaucoma, cataract, and vision loss. Other symptoms may include hearing loss, vitiligo, and neurological and psychiatric disturbances.

Etiology

The exact cause of VKH syndrome is unknown, but it is believed to be an autoimmune response against melanocytes, the cells that produce melanin. This response is thought to be triggered by a viral infection or other environmental factors in genetically susceptible individuals. The syndrome is more common in individuals of Asian, Hispanic, and Native American descent.

Clinical Presentation

Patients with VKH syndrome typically present with a sudden onset of symptoms, which may include severe headache, nausea, and vertigo, followed by visual disturbances such as blurred vision and sensitivity to light. Examination of the eyes may reveal inflammation of the uvea, the middle layer of the eye, and other ocular abnormalities. Other symptoms may include tinnitus, hearing loss, and skin changes such as vitiligo and alopecia.

Diagnosis

Diagnosis of VKH syndrome is based on clinical findings and may be supported by imaging studies such as MRI and ultrasound of the eyes. Laboratory tests may show increased levels of certain inflammatory markers. There are no specific diagnostic criteria for VKH syndrome, but the presence of bilateral uveitis along with neurological and auditory symptoms is highly suggestive of the disease.

Treatment

Treatment of VKH syndrome is aimed at suppressing the immune response and reducing inflammation. This is typically achieved with high-dose corticosteroids, either orally or intravenously. Other immunosuppressive drugs may be used in severe cases or if the disease does not respond to corticosteroids. Early treatment is crucial to prevent permanent vision loss and other complications.

Prognosis

The prognosis of VKH syndrome varies depending on the severity of the disease and the response to treatment. With early diagnosis and aggressive treatment, most patients can achieve good control of their symptoms and prevent serious complications. However, some patients may experience recurrent episodes of inflammation and may require long-term immunosuppressive therapy.

NIH genetic and rare disease info

• NIH info on Vogt–Koyanagi–Harada syndrome

Vogt–Koyanagi–Harada syndrome is a rare disease.

Vogt–Koyanagi–Harada syndrome Resources
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