Vogt-Koyanagi-Harada Syndrome is a rare disease that affects tissues that contain melanocytes, such as the eyes, central nervous system, ear and skin, causing inflammation in the retina of the eye, often associated with dermatological and hearing problems.
This syndrome occurs mainly in young adults between the ages of 20 and 40, with women being the most affected. Treatment consists of the administration of corticosteroids and immunomodulators.
What causes
The cause of the disease is not yet known, but it is believed that it is an autoimmune disease, in which there is aggression on the surface of the melanocytes, promoting an inflammatory reaction with a predominance of T lymphocytes.
Possible symptoms
The symptoms of this syndrome depend on the stage you are in:
Prodromal stage
At this stage, systemic symptoms similar to flu-like symptoms appear, accompanied by neurological symptoms that last only a few days. The most common symptoms are fever, headache, meningism, nausea, dizziness, pain around the eyes, tinnitus, generalized muscle weakness, partial paralysis on one side of the body, difficulty in articulating words correctly or perceiving language, photophobia, tearing, skin and scalp hypersensitivity.
Uveitis stage
At this stage, ocular manifestations predominate, such as inflammation of the retina, decreased vision and eventually detachment of the retina. Some people may also experience hearing symptoms such as tinnitus, pain and discomfort in the ears.
Chronic stage
At this stage, ocular and dermatological symptoms are manifested, such as vitiligo, depigmentation of the eyelashes, eyebrows, which can last from months to years. Vitiligo tends to be symmetrically distributed over the head, face and trunk, and may be permanent.
Recurrence stage
At this stage people can develop chronic inflammation of the retina, cataracts, glaucoma, choroidal neovascularization and subretinal fibrosis.
How the treatment is done
Treatment consists of the administration of high doses of corticosteroids such as prednisone or prednisolone, especially in the acute phase of the disease, for at least 6 months. This treatment can cause resistance and liver dysfunction and in these cases it is possible to choose the use of betamethasone or dexamethasone.
In people whose side effects of corticosteroids make their use in minimally effective doses unsustainable, immunomodulators such as cyclosporine A, methotrexate, azathioprine, tacrolimus or adalimumab can be used, which have been used with good results.
In cases of resistance to corticosteroids and in people who also do not respond to immunomodulatory therapy, intravenous immunoglobulin can be used.
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