Vogt-Koyanagi-Harada Syndrome: Perspectives for Immunogenetics, Multimodal Imaging and Therapeutic Options

Publication date: Available online 7 April 2016
Source:Autoimmunity Reviews
Author(s): Sukhum Silpa-archa, Narumol Silpa-archa, Janine M. Preble, C. Stephen Foster
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 to not only update comprehensive knowledge regarding VKH, but also aims 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 are also provided.



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