Vogt-Koyanagi-Harada syndrome is a rare disease that affects tissues containing melanocytes, such as the eyes, central nervous system, ear and skin, causing inflammation of the retina of the eye, often associated with problems dermatological and auditory.
This syndrome occurs mainly in young adults between the age of 20 and 40, with women being the most affected; treatment involves the administration of corticosteroids and immunomodulators.
- The cause of the disease is not yet known.
- But it is believed to be an autoimmune disease.
- In which there is aggression on the surface of melanocytes.
- Promoting an inflammatory reaction with predominance of T cells.
Symptoms of this syndrome depend on the stage you’re in
Prodrosomal stage
At this stage there are systemic flu symptoms, accompanied by neurological symptoms that last only a few days, being the most common symptoms fever, headache, meningism, nausea, dizziness, pain around the eyes, tinnitus, generalized muscle weakness, partial paralysis on one side of the body, difficulty correctly articulating words or perceiving language, photophobia, tearful eyes, hypersensitivity of the skin and scalp.
Uveite stage
At this stage, eye manifestations predominate, such as inflammation of the retina, decreased vision and possibly retinal detachment; some people may also experience hearing symptoms such as tinnitus, pain and discomfort in the ears.
Chronic stadium
At this stage there are eye and dermatological symptoms, such as vitiligo, eyelash hair removal, eyebrows, which can last from months to years. Vitiligo tends to distribute symmetrically over the head, face and trunk and can be permanent.
Recreation stage
At this stage, people may develop chronic inflammation of the retina, cataracts, glaucoma, choroid neovascularization and subretinian fibrosis.
Treatment involves 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 may lead to resistance and hepatic dysfunction and in these cases the use of betamethasone or dexamethasone may be opted.
In people whose side effects of corticosteroids make their use at minimal doses ineffective and unsustainable, immunomodulators such as cyclosporine A, methotrexate, azathioprine, tacrolimus or adalimumab, which have been used with good results, may be used.
In cases of corticosteroid resistance and in people who do not respond to immunomodulatory treatment, intravenous immunoglobulin may be used.