Vogt-Koyanagi-Harada disease: review of a rare autoimmune disease targeting antigens of melanocytes

Autor(es)

Lavezzo, Marcelo Mendes ^[1] ; Sakata, Viviane Mayumi ^[1] ; Morita, Celso ^[1] ; Caso Rodriguez, Ever Ernesto ^[1] ; Abdallah, Smairah Frutuoso ^[1] ; da Silva, Felipe T. G. ^[1] ; Hirata, Carlos Eduardo ^[1] ; Yamamoto, Joyce Hisae ^[1]

Afiliação do(s) autor(es)

^[1] Univ Sao Paulo, Fac Med, Hosp Clin, Uveitis Serv, Dept Ophthalmol, Rua Diana, 863 Apto 91J, BR-05019000 Sao Paulo - Brazil

• Tipo de documento: Artigo de Revisão
• Fonte: ORPHANET JOURNAL OF RARE DISEASES; v. 11, MAR 24 2016.
• Citações Web of Science: 21

Resumo

Vogt-Koyanagi-Harada disease (VKHD) is a rare granulomatous inflammatory disease that affects pigmented structures, such as eye, inner ear, meninges, skin and hair. This disease is mainly a Th1 lymphocyte mediated aggression to melanocytes after a viral trigger in the presence of HLA-DRB1{*}0405 allele. The absence of ocular trauma or previous intraocular surgery sets VKHD appart from sympathetic ophthalmia, its main differential diagnosis. The disease has an acute onset of bilateral blurred vision with hyperemia preceded by flu-like symptoms. The acute uveitic stage is characterized by a diffuse choroiditis with serous retinal detachment and optic disc hyperemia and edema. Fluorescein angiography in this phase demonstrates multiple early hyperfluorescent points. After the acute uveitic stage, ocular and integumentary system pigmentary changes may appear. Ocular findings may be accompanied by lymphocytic meningitis, hearing impairment and/or tinnitus in a variable proportion of patients. Prompt diagnosis followed by early, aggressive and long-term treatment with high-dose corticosteroids is most often ensued by good visual outcomes. However, some patients may experience chronic uveal inflammation with functional eye deterioration. The current review discusses the general features of VKHD, including epidemiology, classification into categories, differential diagnosis and current therapeutic approaches. (AU)

• Processo FAPESP: 11/50936-7 - Doença de Vogt-Koyanagi-Harada: parâmetros para tratamento efetivo na fase aguda e no estágio tardio
• Beneficiário: Joyce Hisae Yamamoto Takiuti
• Modalidade de apoio: Auxílio à Pesquisa - Regular
• Processo FAPESP: 13/07462-0 - Análise integrada de parâmetros clínicos, funcionais e estruturais na fase aguda e no estágio tardio da doença de Vogt-Koyanagi-Harada: estudo longitudinal
• Beneficiário: Smairah Frutuoso Abdallah
• Modalidade de apoio: Bolsas no Brasil - Programa Capacitação - Treinamento Técnico
• Processo FAPESP: 11/19194-4 - Doença de Vogt-Koyanagi-Harada: parâmetros para tratamento efetivo na fase aguda e no estágio tardio
• Beneficiário: Viviane Mayumi Sakata
• Modalidade de apoio: Bolsas no Brasil - Doutorado Direto
• Processo FAPESP: 14/01222-0 - Análise integrada de parâmetros clínicos, funcionais e estruturais na fase aguda e no estágio tardio da doença de Vogt-Koyanagi-Harada: Estudo longitudina
• Beneficiário: Smairah Frutuoso Abdallah
• Modalidade de apoio: Bolsas no Brasil - Programa Capacitação - Treinamento Técnico