title: Vogt-Koyanagi-Harada disease: review of a rare autoimmune disease targeting antigens of melanocytes.
abstract: 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.
authors.affiliation: Uveitis Service, Department of Ophthalmology, Hospital das Clínicas, Faculdade de Medicina da Universidade de São Paulo, Rua Diana, 863 apto 91J, 05019-000, São Paulo, Brazil.
authors.forename: Marcelo Mendes
authors.initials: MM
authors.lastname: Lavezzo
authors.affiliation: Uveitis Service, Department of Ophthalmology, Hospital das Clínicas, Faculdade de Medicina da Universidade de São Paulo, Rua Diana, 863 apto 91J, 05019-000, São Paulo, Brazil.
authors.forename: Viviane Mayumi
authors.initials: VM
authors.lastname: Sakata
authors.affiliation: Uveitis Service, Department of Ophthalmology, Hospital das Clínicas, Faculdade de Medicina da Universidade de São Paulo, Rua Diana, 863 apto 91J, 05019-000, São Paulo, Brazil.
authors.forename: Celso
authors.initials: C
authors.lastname: Morita
authors.affiliation: Uveitis Service, Department of Ophthalmology, Hospital das Clínicas, Faculdade de Medicina da Universidade de São Paulo, Rua Diana, 863 apto 91J, 05019-000, São Paulo, Brazil.
authors.forename: Ever Ernesto Caso
authors.initials: EE
authors.lastname: Rodriguez
authors.affiliation: Uveitis Service, Department of Ophthalmology, Hospital das Clínicas, Faculdade de Medicina da Universidade de São Paulo, Rua Diana, 863 apto 91J, 05019-000, São Paulo, Brazil.
authors.forename: Smairah Frutuoso
authors.initials: SF
authors.lastname: Abdallah
authors.affiliation: Uveitis Service, Department of Ophthalmology, Hospital das Clínicas, Faculdade de Medicina da Universidade de São Paulo, Rua Diana, 863 apto 91J, 05019-000, São Paulo, Brazil.
authors.forename: Felipe T G
authors.initials: FT
authors.lastname: da Silva
authors.affiliation: Uveitis Service, Department of Ophthalmology, Hospital das Clínicas, Faculdade de Medicina da Universidade de São Paulo, Rua Diana, 863 apto 91J, 05019-000, São Paulo, Brazil.
authors.forename: Carlos Eduardo
authors.initials: CE
authors.lastname: Hirata
authors.affiliation: Uveitis Service, Department of Ophthalmology, Hospital das Clínicas, Faculdade de Medicina da Universidade de São Paulo, Rua Diana, 863 apto 91J, 05019-000, São Paulo, Brazil. joycehy@uol.com.br.
authors.forename: Joyce Hisae
authors.initials: JH
authors.lastname: Yamamoto
mesh_terms: D000818:Animals; D001327:Autoimmune Diseases / Q000276:immunology / Q000378:metabolism*; D006801:Humans; D008544:Melanocytes / Q000276:immunology / Q000378:metabolism*; D035583:Rare Diseases / Q000276:immunology / Q000378:metabolism*; D014605:Uveitis / Q000276:immunology / Q000378:metabolism; D014607:Uveomeningoencephalitic Syndrome / Q000276:immunology / Q000378:metabolism*
keywords: Review; Uveitis; Vogt-Koyanagi-Harada disease