Síndrome de Vogt-Koyanagi-Harada e o seu acometimento multissistêmico

Abstract

Vogt-Koyanagi-Harada's syndrome is a rare disease that affects tissues containing melanocytes, such as the eyes, central nervous system, inner ear and skin. Some ethnic groups have a higher probability of developing the disease, including Asians, Indians and Latin Americans and females are affected more often. Methods: Literature was reviewed in books, articles published on the internet and papers available in the online databases MEDLINE, LILACS and SciELO. Texts were selected that focused on otorhinolaryngological symptoms. Literature review: The disease probably has autoimmune etiology, with aggression occurring on the surface of melanocytes by promoting inflammatory reaction in which T lymphocytes predominate. The allele most often found in association with this disease is HLA DRB1*0405. Clinical manifestations are divided into four stages: prodromal, uveitic, chronic and recurrent. Otorhinolaryngological symptoms occur during the uveitic stage and are characterized by bilateral sensorineural hearing loss, tinnitus and vestibular symptoms. Diagnosis is made according to the diagnostic criteria for the disease. Treatment is primarily with corticosteroids. Conclusions: It is important that professionals in other specialties are able to recognize Vogt-Koyanagi-Harada's syndrome, because late diagnosis can lead to ocular and cutaneous sequelae.

Vogt-Koyanagi-Harada syndrome: Experience in a Belgian university hospital and review of the literature

“…Therefore, a general physician can certainly play an important role in the diagnostic process. [6,10] The age and gender distribution of our patients, with twice as many women as men and a median age of 34 years at diagnosis, is consistent with literature. [17,18] A comparison of extra-ocular symptoms between previous and the present case series is presented in Table 4, showing a higher frequency of extra-ocular manifestations reported in our population.…”

“…The use of intravenous pulse therapy as an initial treatment is still controversial, and has not yet proven to result in better visual acuity afterwards. [3,10,11,13] In our case series patients seemed to reach complete remission more often (80% vs. 42%) when treated with iv steroids initially.…”

“…Intravitreal injections can be applied to treat intra-or subretinal fluid. [10,11] Second or third line immunomodulating therapy (e.g. methotrexate, azathioprine, cyclosporine, rituximab, adalimumab) is given in case of corticosteroid resistance or side effects.…”

“…[1][2][3][4][10][11][12][13][14][15] Ocular complications linked to both disease activity and treatment may lead to permanent visual loss after extinction of the uveitic phase. [3,10] The visual prognosis of VKHS varies and depends mainly on early diagnosis and rapid appropriate treatment. [10] The most important factor associated with poor prognosis is inadequate treatment: start of corticosteroids more than 15 days after onset, at a suboptimal dose or for less than six months.…”

“…[3,10] The visual prognosis of VKHS varies and depends mainly on early diagnosis and rapid appropriate treatment. [10] The most important factor associated with poor prognosis is inadequate treatment: start of corticosteroids more than 15 days after onset, at a suboptimal dose or for less than six months. [16] We performed a retrospective case series review from an internist's viewpoint, focusing on the extra-ocular manifestations in patients presenting with VKHS in a Belgian referral hospital.…”