Vogt-Koyanagi-Harada disease.
Vogt-Koyanagi-Harada (VKH) disease affects primarily persons who are Asian, Latino, Native American, or Asian Indian. Women appear to be affected more commonly than men, and VKH disease may occur at all ages, including childhood. Experimental data continue to support an autoimmune etiology for VKH disease, directed most probably against an antigenic component of the melanocyte, possibly tyrosinase or a tyrosinase-related protein. The clinical diagnosis of VKH disease continues to be based on physical findings. Newer imaging modalities such as magnetic resonance imaging, indocyanine green angiography, and digital image analysis have not added appreciably to our understanding of the condition. First-line therapy consists of high-dose corticosteroids, with use of corticosteroid-sparing agents for resistant or recalcitrant disease. Complications are the main cause of reversible and irreversible vision loss, with subretinal fibrosis and choroidal neovascular membranes having particularly poor prognoses.
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Department of Ophthalmology, Doheny Eye Institute, Pathology, University of Alabama at Birmingham, Birmingham, 700 18th Street South, EFH 6001 Alabama, USA. rwr@uab.edu
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D015551:Autoimmunity / Q000276:immunology; D001766:Blindness / Q000175:diagnosis / Q000209:etiology; D020256:Choroidal Neovascularization / Q000150:complications / Q000175:diagnosis; D005451:Fluorescein Angiography; D005654:Fundus Oculi; D005938:Glucocorticoids / Q000627:therapeutic use; D006801:Humans; D008279:Magnetic Resonance Imaging; D008544:Melanocytes / Q000201:enzymology / Q000276:immunology; D008562:Membrane Glycoproteins; D014442:Monophenol Monooxygenase / Q000276:immunology; D010088:Oxidoreductases / Q000276:immunology; D011379:Prognosis; D011506:Proteins / Q000276:immunology; D014607:Uveomeningoencephalitic Syndrome / Q000150:complications / Q000175:diagnosis / Q000188:drug therapy / Q000276:immunology
D005938:Glucocorticoids; D008562:Membrane Glycoproteins; D011506:Proteins; D010088:Oxidoreductases; C489799:TYRP1 protein, human; C064056:tyrosinase-related protein-1; D014442:Monophenol Monooxygenase