title: Vogt-Koyanagi-Harada syndrome: Perspectives for immunogenetics, multimodal imaging, and therapeutic options.
abstract: Vogt-Koyanagi-Harada syndrome (VKH) is a bilateral, diffuse granulomatous uveitis associated with neurological, audiovestibular, and dermatological systems. The primary pathogenesis is T-cell-mediated autoimmune response directed towards melanocyte or melanocyte-associated antigens causing inflammation of the choroidal layer. This phenomenon usually leads to diffuse inflammatory conditions throughout most parts of eye before ocular complications ensue. The diagnosis is achieved mainly by clinical features according to the revised diagnostic criteria of VKH published in 2001, without confirmatory serologic tests as a requirement. However, ancillary tests, especially multimodal imaging, can reliably provide supportive evidence for the diagnosis of early cases, atypical presentations, and evaluation of management. Prompt treatment with systemic corticosteroids and early non-steroidal immunosuppressive drug therapy can lessen visually threatening ocular complications and bring about good visual recovery. Close monitoring warrants visual stabilization from disease recurrence and ocular complications. This article review aims not only to update comprehensive knowledge regarding VKH but also to emphasize three major perspectives of VKH: immunogenetics as the major pathogenesis of the disease, multimodal imaging, and therapeutic options. The role of anti-vascular endothelial growth factor therapy and drug-induced VKH is also provided.
authors.affiliation: Faculty of Medicine, Department of Ophthalmology, Rajavithi Hospital, 2 Phayathai Road, Ratchathewi District, Bangkok 10400, Thailand. Electronic address: sukhumsilp@gmail.com.
authors.forename: Sukhum
authors.initials: S
authors.lastname: Silpa-Archa
authors.affiliation: Faculty of Medicine, Department of Dermatology, Siriraj Hospital, 2 Wanglang Road, Bangkoknoi, Bangkok 10700, Thailand. Electronic address: doctornarumol@gmail.com.
authors.forename: Narumol
authors.initials: N
authors.lastname: Silpa-Archa
authors.affiliation: Wayne State University School of Medicine, 540 E Canfield St., Detroit, MI 48201, USA. Electronic address: Jpreble22@gmail.com.
authors.forename: Janine M
authors.initials: JM
authors.lastname: Preble
authors.affiliation: Massachusetts Eye Research and Surgery Institution, 1440 Main Street, Suite 20, Waltham, MA 02451, USA; Ocular Immunology and Uveitis Foundation, Waltham, MA, USA; Harvard Medical School, Boston, MA, USA. Electronic address: sfoster@mersi.com.
authors.forename: C Stephen
authors.initials: CS
authors.lastname: Foster
mesh_terms: D015551:Autoimmunity; D006801:Humans; D007125:Immunogenetics; D007166:Immunosuppressive Agents / Q000627:therapeutic use*; D064847:Multimodal Imaging; D014607:Uveomeningoencephalitic Syndrome / Q000188:drug therapy / Q000235:genetics / Q000276:immunology* / Q000473:pathology*; D042461:Vascular Endothelial Growth Factor A / Q000037:antagonists & inhibitors
chemical_list: D007166:Immunosuppressive Agents; D042461:Vascular Endothelial Growth Factor A
keywords: Immunogenetic; Immunosuppressive drug therapy; Multimodal imaging; Uveitis; Vitiligo; Vogt–Koyanagi–Harada syndrome