Vogt-Koyanagi-Harada syndrome is a rare disease that affects melanocyte-containing tissues 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 administration of corticosteroids and immunomodulators.
What causes
The cause of the disease is not yet known, but it is believed to be an autoimmune disease, in which aggression occurs to the surface of melanocytes, promoting inflammatory reaction with a predominance of T lymphocytes.
Possible symptoms
The symptoms of this syndrome depend on the stage in which it is found:
Prodromic stage
At this stage, there are systemic symptoms similar to an influenza-like illness accompanied by neurological symptoms that last only a few days. The most common symptoms are fever, headache, meningism, nausea, vertigo, pain around the eyes, tinnitus, generalized muscle weakness, partial paralysis of one side of the body, difficulty in articulating words correctly or perceiving language, photophobia, lacrimation, cutaneous and scalp hypersensitivity.
Uveitis stage
At this stage, ocular manifestations such as inflammation of the retina, impaired vision and eventually retinal detachment predominate. Some people may also have auditory symptoms such as tinnitus, pain, and discomfort in the ears.
Chronic stage
At this stage ocular and dermatological symptoms, such as vitiligo, depigmentation of the eyelashes, eyebrows, can last from months to years. The vitiligo tends to distribute itself symmetrically by the head, face and trunk, being able to be permanent.
Recurrence stage
At this stage people may develop chronic inflammation of the retina, cataracts, glaucoma, choroidal neovascularization, and sub-retinal fibrosis.
How is the treatment done?
Treatment consists of administration of high doses of corticoids such as prednisone or prednisolone, especially in the acute phase of the disease, for at least 6 months. This treatment can cause hepatic resistance and dysfunction and in these cases one can choose to use betamethasone or dexamethasone.
In people in whom side effects of corticosteroids render their use useless at minimally effective doses, immunomodulators such as cyclosporin A, methotrexate, azathioprine, tacrolimus, or adalimumab may 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 may be used.
