Vogt-Koyanagi-Harada's disease: literature review

International Archives of Otorhinolaryngology 01/2008;
Source: DOAJ

ABSTRACT 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. Objective: To review in the literature the several aspects of Vogt-Koyanagi-Harada's disease focusing on its cause and also to study its otolaryngology aspects through online databases Cochrane, LILACS, MEDLINE, OMIM e SciELO. Literature Review: This exact cause remains unknown, but there are evidences of an autoimmune process against an antigenic component on the melanocyte configuring an inflammatory T-cell-mediated immune response. The HLADRB1* 0405 allele is the one that is most associated to the disease. The clinic manifestations are divided in four: prodromal, uveitic, chronic and recurrence stages. The otolaryngology's aspects occur at the uveitic stage. Hearing loss is sensorineural bilateral and quickly progressive and could be associated with tinnitus. The vestibular component is less affected with vertigo, nystagmus and abnormal vestibular reflex. The otolaryngology's manifestations do not influence the recurrences or complications and they also have great prognosis with the treatment established. The diagnosis of this syndrome is based on clinical criteria, but audiological test could help. The therapy is corticosteroid-based. Final Comments: The most important factor for prognosis is the immediately treatment. The rarity of this syndrome makes its diagnosis a challenge and the presence of deafness, tinnitus and vertigo must consider its diagnosis.

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    ABSTRACT: Vogt-Koyanagi-Harada (VKH) syndrome is a systemic condition characterized by ocular inflammatory disease as well as skin, ear, and meningeal manifestations. Patients with VKH often report tinnitus and hearing loss, but these symptoms tend to be given secondary consideration because most undergo treatment with steroids to prevent blindness resulting from granulomatous uveitis, exudative retinal detachment, and optic nerve inflammation. METHODS/STUDY DESIGN: In the current retrospective review, 24 patients with this syndrome were screened for auditory system abnormalities. All patients denied history of noise exposure or ototoxic agent exposure. The age range of the patients was 13 to 42 years. Three patients reported tinnitus and two patients reported sudden hearing loss. One patient experienced vertigo and aural fullness. Eight of 24 patients had pure-tone thresholds greater than 25 dB hearing loss at two or more frequencies. Five of 24 of these patients experienced hearing loss outside of the 95% confidence interval for published age-matched control populations. There was sloping sensorineural hearing loss at 4 kHz and above in five of 24 patients. All eight patients with hearing loss experienced some degree of hearing loss at 4 kHz or above. Three patients had mild to moderate low-frequency sensorineural hearing loss. There were no tympanometric abnormalities suggestive of conductive involvement. Abnormal acoustic reflex decay was observed in one patient. We conclude that a significant number of patients with VKH experience sensorineural hearing loss and that every patient with VKH should undergo a review of systems for auditory abnormalities and referral for audiologic testing if symptomatic. It is possible that untreated patients may experience worse symptoms.
    The Laryngoscope 11/2006; 116(10):1873-6. DOI:10.1097/01.mlg.0000234946.31603.fe · 2.03 Impact Factor
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    ABSTRACT: Uveomeningoencephalitides, commonly known as Vogt-Koyanagi-Harada (VKH) disease, are characterized by chronic bilateral granulomatous panuveitis involving the central nervous, auditory and integumentary systems. Visual prognosis is generally favourable, but outcomes in patients with VKH disease may vary. Also, the treatment of choice differs in different parts of the world. This review addresses the literature on the possible pathogenesis, diagnosis and treatment of this disorder. Atypical presentations of VKH disease, as well as those associated with interferon-alpha therapy, have been reported. Most reports suggest an association with autoimmunity. The diagnostic criteria were revised by the International Workshop on VKH in 1999, allowing for the presence of different ocular findings in the early and late stages of the disease. New techniques have also been developed to aid in the rapid diagnosis of VKH disease and evaluation of treatment. Different routes of administration of corticosteroid and adjuvant therapy were tried, with positive results. Although the pathogenesis of VKH disease is uncertain and antigen-specific treatment strategies have not yet been developed, reports increasingly suggest an autoimmune nature for uveomeningoencephalitides. Currently, systemic corticosteroid therapy remains the standard initial treatment. Different routes of administration are used to reduce the frequency of side effects of systemic corticosteroids, and there are various adjuvant therapies. With the aid of modern equipment, early diagnosis and prompt and appropriate treatment, resulting in better visual outcomes, can be anticipated. A large-scale, multinational, prospective study is warranted to determine the optimal initial therapy.
    Current Opinion in Neurology 07/2005; 18(3):323-9. DOI:10.1097/01.wco.0000169753.31321.4e · 5.73 Impact Factor
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    ABSTRACT: OBJETIVO: Avaliar o uso da injeção intravítrea do acetato de triancinolona no tratamento da fase aguda da síndrome de Vogt-Koyanagi-Harada, demonstrando a rápida resolução do descolamento seroso de retina. MÉTODOS: Nove olhos de cinco pacientes apresentando descolamento seroso de retina associado à síndrome de Vogt-Koyanagi-Harada foram tratados com uma única injeção intravítrea de 4 mg de acetato triancinolona. Os seguintes parâmetros foram avaliados: melhor acuidade visual, pressão intra-ocular e a altura do descolamento de acordo com a tomografia de coerência óptica. RESULTADOS: Em todos os olhos a tomografia de coerência óptica revelou diminuição marcada no descolamento seroso de retina na primeira semana após a injeção intravítrea do acetato de triancinolona, com subseqüente recuperação da acuidade visual e das características anatômicas retinianas normais. Não foram observadas complicações durante o seguimento, que variou de 5 a 12 meses (média de 7,8 meses). CONCLUSÕES: A injeção intravítrea do acetato de triancinolona pode proporcionar em curto tempo a resolução do processo inflamatório e exsudativo intra e sub-retiniano na síndrome de Vogt-Koyanagi-Harada cursando com melhora da acuidade visual. São necessários novos estudos para avaliar a eficácia e a segurança deste tipo de procedimento a longo prazo.
    Arquivos Brasileiros de Oftalmologia 01/2004; DOI:10.1590/S0004-27492004000300005 · 0.44 Impact Factor

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