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Background: Vogt-Koyanagi-Harada syndrome (VKH) is a systemic disease that affects organs profuse in melanocytes, presenting with a chronic and diffuse bilateral granulomatous panuveitis, as well as neurological, auditory, and cutaneous manifestations. In this article, a systematic approach is presented for the diagnostic management of VKH syndrome, considering relevant diagnostic possibilities to rule out other entities that manifest similar symptoms. Clinical case: 71-year-old man with a long-standing history of vitiligo, who experienced visual loss in his right eye 6 months before his admission, along with bilateral hearing loss predominantly in the right ear. During his hospitalization, he presented with chronic headache, fever, and significant involuntary weight loss. Ophthalmological examination revealed that his right eye only perceived light and had hyperemic bulbar conjunctiva, while the left eye had a visual acuity of 20/200. The right fundus had scattered pigmentation, while the left had an edematous optic disc and right optic atrophy. Conclusions: The diagnosis of VKH syndrome is established by the presence of the 5 diagnostic criteria for complete disease, including retinal depigmentation, neurological alterations, and dermatological manifestations. Since patients can present with a wide variety of symptoms, initial differential diagnosis should be considered, which represents a diagnostic challenge.

Introducción: el síndrome de Vogt-Koyanagi-Harada (VKH) es una enfermedad sistémica que afecta a órganos ricos en melanocitos y se manifiesta con una panuveítis granulomatosa bilateral, crónica y difusa, así como con manifestaciones neurológicas, auditivas y cutáneas. En este artículo se presenta un enfoque sistemático para el abordaje diagnóstico del síndrome de VKH y se consideran las posibilidades diagnósticas relevantes para descartar otras entidades que se presentan con síntomas similares. Caso clínico: hombre de 71 años con antecedentes de vitiligo de larga data, quien experimentó una pérdida visual en su ojo derecho seis meses antes de su ingreso, junto con hipoacusia bilateral, predominantemente en el oído derecho. Durante su hospitalización, presentó cefalea crónica, fiebre y una significativa pérdida involuntaria de peso. En la exploración oftalmológica, el ojo derecho solo percibía luz y presentaba conjuntiva bulbar hiperémica, mientras que el ojo izquierdo tenía una agudeza visual de 20/200. El fondo del ojo derecho presentaba pigmentación dispersa, mientras que el izquierdo tenía una papila edematosa y atrofia óptica derecha. Conclusiones: el diagnóstico del síndrome de VKH se establece mediante la presencia de los 5 criterios diagnósticos para la enfermedad completa, incluida la hipopigmentación retiniana, las alteraciones neurológicas y las manifestaciones dermatológicas. Dado que los pacientes pueden presentar una amplia variedad de síntomas, el diagnóstico diferencial debe considerarse inicialmente, lo que representa un desafío diagnóstico.

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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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ABSTRACT Vogt-Koyanagi-Harada (VKH) syndrome is an inflammatory condition of unknown etiology that can affect the eye. The most common ocular manifestation related to VKH is bilateral diffuse uveitis associated to exudative retinal detachment. Although these patients respond well to steroid pulse therapy, we report a case of a 44-year-old female patient presenting bilateral exudative retinal detachment and clinical diagnosis of VKH, who did not respond to the first cycle of 3-day pulse therapy with methylprednisolone. The exudation was reabsorbed only after a second cycle of steroid therapy.

RESUMO A doença de Vogt-Koyanagi-Harada é inflamatória e de etiologia desconhecida, podendo afetar o olho. A manifestação ocular mais comum relacionada à doença de Vogt-Koyanagi-Harada é a uveíte difusa bilateral associada ao descolamento exsudativo da retina. Embora esses pacientes respondam bem à pulsoterapia com esteroides, relatamos um caso de paciente de 44 anos que apresentou descolamento exsudativo bilateral da retina com diagnóstico clínico de doença de Vogt-Koyanagi-Harada que não respondeu ao primeiro ciclo de pulsoterapia de 3 dias com metilprednisolona. A exsudação apenas reabsorveu após uma segunda rodada de terapia com esteroides.

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Purpose To evaluate the characteristics of Vogt-Koyanagi-Harada (VKH) syndrome in Brazilian children.Methods Clinical data were obtained from the medical records of six children with VKH disease from March 2014 to June 2018 at the Federal University of São Paulo, Brazil.Results Six patients met the diagnostic criteria for VKH. The patients, who ranged in age from 5 to 8 years, all presented with chronic disease. The most common ocular finding was abnormal fundus pigmentation in five patients followed by band keratopathy in four. Two patients had glaucoma, cataract, and subretinal neovascularization. The final visual acuity was less than 20/40 in 50% of the eyes.Conclusion Visual outcomes were favorable in half of patients depending on the long-term sequelae. VKH is rarely reported in children and it may be sight-threatening, and requires careful attention, being an important differential diagnosis.

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PURPOSE: To describe the pattern of uveitis in a tertiary center in Santiago, Chile. METHODS: We reviewed all of the case records with a presumptive diagnosis of uveitis from patients referred to the Uveitis Department of Hospital del Salvador between 2002 and 2012. Initial assessment was standardized. We tested for association among causes, gender, and age groups. RESULTS: Anterior uveitis was the leading anatomical location of the inflammation (40.4%). A specific etiology was demonstrated in 59% of cases (28.7% infectious). Vogt-Koyanagi-Harada syndrome (VKH) was the most frequent cause of uveitis (17.2%). We found association between idiopathic intermediate uveitis, toxocariasis, juvenile idiopathic arthritis, VKH, toxoplasmosis, diabetes mellitus (DM)-associated uveitis, tuberculosis, and idiopathic anterior uveitis and age groups. VKH, DM-associated uveitis, and syphilis-associated uveitis were associated with gender. CONCLUSIONS: Our sample shows a distribution of causes of uveitis similar to those in developed countries. The prevalence of VKH is higher than expected.

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We report a 24-year-old man with Vogt-Koyanagi-Harada (VKH) syndrome who developed a macular hole (MH) during the acute inflammatory stage. Spontaneous resolution was unlikely because of the MH dimensions and absence of vitreous adherence. The patient underwent pars plana vitrectomy (PPV) and internal limiting membrane peeling during the acute stage followed by retinopexy with octafluoropropane injection and prone positioning for 5 days. The MH closed and the best-corrected visual acuity (BCVA) improved from 20/400 to 20/40. Prompt surgical intervention may be an alternative for treating MHs and obtaining visual recovery in special cases even in the acute inflammatory stage.

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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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Background: Topical and systemic steroids are the first line of treatment of non infectious inflammatory ocular disease. Immunosuppresants are reserved as a second line treatment. Aim: To evaluate the role ofAzathioprine (AZA) as a coadyuvant immunosuppressive treatment for non infectious ocular inflammatory diseases (OIDs) resistant to systemic steroid therapy in a retrospective, noncomparative interventional case series. Patients and methods: Patients using oral Prednisone due to an active or recurrent OID, without clinical response, and not receiving any other immunosuppressive treatment were studied. A standard protocol of oral Prednisone (0.5 mg/kg/ day) and oral AZA (2-3 mg/kg/day) during one year was used. Ocular and systemic monthly evaluations were done including relapse rate, steroid dosage, inflammatory score and visual acuity. Results: Thirty patients (10 male) aged 18-75 years (mean 44 years) were studied. Three had bilateral anterior uveitis, one had pars planitis, four had diffuse uveitis, eight Vogt-Koyanahi-Harada syndrome, three Behget's disease, three necrotizing scleritis and eight had retinochoroidopathy A complete initial response was observed in 26 patients (87 percent). The time of response was between 1 to 6 months (mean 2.65 months). Seventeen percent of these had a relapse 6 to 12 months after AZA was started. In 61 percent, visual acuity improved. The ocular inflammatory score decreased in 86.5 percent. Eleven patients had mild controlled side effects that did not require discontinuation of AZA. Conclusions: Combined systemic steroid and oral AZA therapy is safe and effective in controlling steroid resistant non infectious inflammatory ocular diseases.

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The Vogt-Koyanagi-Harada syndrome have been studied in human beings. A similar syndrome was firstreported in Japan in Akita dogs. Several reports havea ppeared in veterinary literature in Canada, in the United States and in United Kingdom affecting different breeds. We described the clinical aspects of the Vogt-Koyanagi-Harada-Like syndrome in twenty-one Akita dogs. The ocular findings include bilateral anterior or posterior uveitis or a severe panuveitis, cutaneous depigmentation around eyelids, lips, nasal planum, and scrotum which can present ulceration and crusting. The cause of the disease remains unknown and the administration of high doses of corticosteroids is the treatment of choice. This is the first communication of the syndrome in Brazil.

A síndrome de Vogt-Koyanagi-Harada, caracterizada pela associação de uveite e alteraçõe cutaneas e de meninge, de causa desconhecida é bastante estudada na oftalmologia humana. Foi primeiro descrita no Japão uma síndrome semelhante em cães da raça Akita, sendo que relatos em outras raças e outrosçpaíses como Canadá, Estados Unidos e Inglaterra foram feitos posteriormente. Descrevemos pela primeira vez no Brasil esta associação úveo-dermatológica em 21cães da raça Akita. Caracterizada por uveíte e despigmentação ao redor das pálpebras, lábios, narina e escroto podendo haver ulceração com formação de crostas nestas regiões. A administração de altas doses de corticosteróides é o tratamento de eleição.