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El síndrome de Vogt-Koyanagi-Harada es una enfermedad autoinmune multisistémica crónica, caracterizada por panuveítis difusa granulomatosa bilateral con desprendimiento exudativo de retina y papilitis. Compromete el sistema nervioso central (meninges, disacusia neurosensorial) así como piel y mucosas. A pesar de ser una enfermedad compleja y poco frecuente, se hace necesario comprender la importancia del diagnóstico rápido y el tratamiento oportuno con seguimiento especializado. Es por ello que se decidió realizar una revisión de la literatura con el objetivo de actualizar los conocimientos existentes sobre este tema. La búsqueda se realizó en diferentes publicaciones y textos básicos de la especialidad. Las fuentes consultadas fueron las bases de datos PubMed y Google Scholar. El diagnóstico de la enfermedad es esencialmente clínico y son los oftalmólogos quienes más lo sospechan por ser los síntomas oculares los más frecuentes y dramáticos. El pronóstico visual de los pacientes es generalmente bueno si el diagnóstico es precoz y se indica un tratamiento adecuado. Los corticosteroides sistémicos a altas dosis asociados a inmunosupresores y agentes biológicos tienen gran impacto en la evolución de la enfermedad, sobre todo estos últimos a nivel mundial, previniendo complicaciones y permitiendo resultados visuales satisfactorios para una mejor calidad de vida del paciente(AU)

Vogt-Koyanagi-Harada syndrome is a chronic multisystem autoimmune disease characterized by bilateral diffuse granulomatous panuveitis with exudative retinal detachment and papillitis. It involves the central nervous system (meninges, sensorineural dysacusis) as well as skin and mucous membranes. In spite of being a complex and infrequent disease, it is necessary to understand the importance of rapid diagnosis and timely treatment with specialized follow-up. For this reason, it was decided to carry out a review of the literature with the aim of updating the existing knowledge on this subject. The search was carried out in different publications and basic texts of the specialty. The sources consulted were the PubMed and Google Scholar databases. The diagnosis of the disease is essentially clinical and it is the ophthalmologists who suspect it the most because the ocular symptoms are the most frequent and dramatic. The visual prognosis of patients is generally good if the diagnosis is early and adequate treatment is indicated. Systemic corticosteroids at high doses associated with immunosuppressants and biological agents have a great impact on the evolution of the disease, especially the latter worldwide, preventing complications and allowing satisfactory visual results for a better quality of life of the patient(AU)

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The case is presented of a 58-year-old woman with a personal history of metastatic melanoma under treatment with trametinib and dabrafenib, as well as a decrease in bilateral visual acuity. On examination, it was observed that she had an anterior uveitis, vitritis, serous retinal detachment, vasculitis and disc oedema in both eyes. She was diagnosed with a Vogt-Koyanagi-Harada-like syndrome secondary to MAP kinase pathway inhibition. In addition to the withdrawal of the oncology drugs, systemic and topical corticosteroids were prescribed, but this treatment only achieved a partial improvement of the disease when biological therapy was re-introduced. Therefore, a bilateral intravitreal dexamethasone implant was added, which led to a favourable progression in her symptomatology, as well as in the findings of complementary tests. Intraocular inflammation is a complication described after treatment with trametinib and dabrafenib. An accurate diagnosis, added to corticosteroid treatment, systemic and intravitreal, led us to obtain optimal results.

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Resumo Relatamos um caso atípico de uma paciente de 40 anos com apresentação completa da Síndrome de Vogt-Koyanagi-Harada (SVKH) que após 17 anos do diagnóstico inicial evoluiu com descolamento seroso de coroide. A paciente procurou atendimento com queixa de dor em olho esquerdo (OE). O exame oftalmológico revelou acuidade visual (AV) igual a de movimento de mãos, à biomicroscopia foi observada reação inflamatória granulomatosa na câmara anterior, a tonometria foi igual a 0 mmhg, e a fundoscopia indevassável pela pouca midríase e turvação de meios em OE. O descolamento seroso de coroide foi avaliado através de ultrassonografia ocular. A abordagem terapeutica intituida para paciente consistiu em prednisona 1mg/kg/dia via oral, dexametasona 1mg/mL e atropina 1% colírios. A evolução do quadro foi satisfatória, com melhora da AV para 20/40, ausência de reação inflamatória em câmara anterior, normalização da pressão intraocular e resolução do descolamento seroso de coroide em OE. Concluimos que a fase crônica da SVKH, apesar da manifestação classicamente descrita ser uveíte anterior, pode ter outras apresentações e o descolamento seroso da coroide é uma rara complicação.

Abstract We report an atypical case of a 40-year-old woman with complete presentation of Vogt-Koyanagi-Harada Syndrome (VKH) who presented with unilateral serous choroidal detachment 17 years after the diagnosis. The patient complained of pain in the left eye, the ophthalmologic examination revealed visual acuity (VA) equal to hand motion; biomicroscopy revealed a granulomatous inflammatory reaction in the anterior chamber, tonometry was equal to 0 mmhg, and the fundoscopy was impracticable. Serous choroidal detachment was assessed by ocular ultrasonography. The therapeutic approach proposed for the patient consisted of prednisone 1mg / kg / day orally, dexamethasone 1mg / mL and atropine 1% eye drops. The evolution of the condition was satisfactory, with VA improvement to 20/40, absence of inflammatory reaction in anterior chamber, normalization of intraocular pressure and resolution of serous choroidal detachment in OS. We conclude that the chronic phase of VKH, although classically described as anterior uveitis, may have other presentations and the serous choroid detachment is a rare complication.

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Se presenta el caso de una paciente femenina de 28 años de edad, con síndrome de Vogt Koyanagi Harada (SVKH), con síntomas acompañantes de dolor ocular, cefalea y disminución de la visión en ambos ojos y con signos de hipoacusia e irritación meníngea. Se encontró en ella panuveítis granulomatosa bilateral. Más tarde desarrolló presión intraocular alta y síntomas de diabetes secundaria al tratamiento con esteroides. Por esta razón el tratamiento inicial fue cambiado a inmunosupresores. La alta presión intraocular fue controlada con timolol 0,5 por ciento tópico. La ciclosporina A mostró buena eficacia clínica y remisión de la inflamación ocular. Pacientes con intolerancia y/o resistencia a los esteroides requieren inmunosupresores (AU)

This is a case of a female 28-year-old patient suffering from Vogt Koyanagi Harada syndrome (VKHS), with symptoms of eye pain, headache and low vision of both eyes and signs of hypoacusis and meningeal irritation. Eye examination showed bilateral granulomatous pan-uveitis. Later she developed high intraocular pressure and symptoms of diabetes, secondary to the steroid treatment. Therefore, the initial treatment was changed to immunosuppressive treatment. The high intraocular pressure was controlled with topical timolol 0, 5 percent eyedrops. Cyclosporine A showed a good clinical efficacy and remission of the ocular inflammation. Patients who suffered from intolerance and/or resistance to corticosteroids required immunosuppressive therapy (AU)

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Se presenta el caso de un paciente de 43 años de edad, sexo masculino, procedencia rural, el cual acude por mala visión bilateral y sordera. Se le diagnostica síndrome de Vogt Koyanagi Harada. Se realiza cirugía de catarata con implante de lente intraocular en cámara posterior en ojo derecho. Se analizan los resultados del examen oftalmológico y clínico general realizados, además de los estudios de laboratorio. Se comparan nuestros resultados con los informados por otros autores de la literatura médica nacional e internacional(AU)

A case is presented, a patient with 43 years old, male, rural origin, with bilateral low vision and deafness. Syndrome is diagnosed with Vogt Koyanagi Harada. Cataract surgery was applied with implantation of posterior chamber intraocular lens in the right eye. Results about clinical and ophthalmology test were analyzed in addition to laboratory studies. Results were compared with those reported by other authors of the national and international medical literature (AU)

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A síndrome de Vogt-Koyanagi-Harada é uma doença inflamatória, multissistêmica, rara e autoimune contra proteínas da membrana dos melanócitos, responsáveis pela produção de melanina. Afeta, portanto, estruturas pigmentares, como retina, meninges, ouvido interno, pele e sistema nervoso central. Acomete preferencialmente, mulheres de peleescura ou miscigenadas, na quarta década de vida, e caracteriza-se por uveíte bilateral, principalmente posterior, hipoacusia neurossensorial bilateral, meningismo e alterações de pele, como vitiligo. O diagnóstico é essencialmente clínico, apoiado em achados à retinografia, ultrassonografia ocular e angiografia ocular fluoresceínica. O tratamentobaseia-se em corticoterapia precoce e agressiva, seguida de imunomoduladores, como a ciclosporina, nos casos recidivantes ou refratários. O prognóstico apresenta relação com o tempo entre o diagnóstico e o início do tratamento. As principais complicações são as oculares, como catarata, glaucoma, membrana neovascular subretiniana e fibrose subretiniana.

Vogt-Koyanagi-Harada disease is an inflammatory, multisystemic, rare autoimmune disease against membrane proteins of melanocytes that are responsible for the production of melanin, and therefore affect pigmented structures such as retina, meninges, inner ear, skin and central nervous system. Mainly affects women of dark skin or blended, in the 4th decade of life, and is characterized by bilateral, mainly posterior, uveitis, bilateral sensorineural hearing loss, meningism and integumentary findings, such as vitiligo. The diagnostic is essencially clinical, supported byretinography, ultrasonography and fluorescein angiography. The therapy relies on early and aggressive treatment with systemic corticosteroids, followed by immunomodulatory therapy, such as cyclosporine, in relapsed or refractory cases. The prognosis is correlated with the time between diagnosis and initiation of treatment. The main complicationsare the ocular ones, such as cataract, glaucoma, subretinal neovascular membrane and subretinal fibrosis.

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Introdução: A síndrome de Vogt-Koyanagi-Harada é uma doença rara que atingem tecidos contendo melanócitos como nos olhos, sistema nervoso central, pele e ouvido interno. Apresenta predominância nos asiáticos, indianos e latinoamericanos e no sexo feminino. Objetivo: Realizar uma revisão de literatura sobre os diversos aspectos da Síndrome de Vogt-Koyanagi-Harada, enfatizando a sua etiopatogenia bem como as manifestações clínicas otorrinolaringológicas através dos bancos de dados on line Cochrane, LILACS, MEDLINE, OMIM e SciELO. Revisão de Literatura: A etiologia é incerta, mas há indícios de processo auto-imune contra antígenos na superfície dos melanócitos determinando uma resposta inflamatória imune com predomínio de linfócitos T. O alelo HLA-DRB1*0405 é o mais associado à doença. As manifestações clínicas são divididas em quatro estágios: prodrômico, uveítico, crônico e de recorrência. As manifestações otorrinolaringológicas se desenvolvem na fase uveítica. Há hipoacusia do tipo neurossensorial bilateral e rapidamente progressiva associada a zumbidos. O comprometimento do componente vestibular leva a vertigem, nistagmo horizontal e alteração do reflexo óculo-vestibular.

Introduction: Vogt-Koyanagi-Harada's disease is a rare syndrome that affects tissues with melanocytes like eyes, central nervous system, skin and inner ear. It affects primarily Asians, Indians and Latin Americans and also women.

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