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Vogt-Koyanagi-Harada Disease (Uveomeningitic Syndrome)

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Albert and Jakobiec's Principles and Practice of Ophthalmology

Abstract

Vogt-Koyanagi-Harada (VKH) disease is a systemic disease with characteristic ocular and extraocular findings. It can manifest in four stages: prodromal, acute uveitic, chronic, and chronic recurrent stages. Symptoms in the prodromal stage include fever, headache, vertigo, nausea, meningismus, dysacusia, and tinnitus. This stage typically lasts a few days. The acute uveitic stage is characterized by bilateral granulomatous panuveitis. Chronic stage manifestations include vitiligo, alopecia, and poliosis, as well as choroidal depigmentation. In the chronic recurrent stage, there may be repeated episodes of usually anterior uveitis, often leading to visually debilitating sequelae. The diagnosis of VKH is a clinical one, supported by ancillary testing, such as fluorescein angiography, optical coherence tomography (OCT), fundus autofluorescence (FAF), indocyanine green angiography, ultrasonography, and lumbar puncture. Initial treatment consists of intravenous or oral high-dose steroids followed by a prolonged taper. Steroid-sparing immunomodulatory therapy can be effective in improving visual outcomes and reducing recurrent rates.

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Authors and Affiliations

  1. Department of Ophthalmology, Roski Eye Institute, Keck School of Medicine of USC, University of Southern California, Los Angeles, CA, USA

    Youning Zhang, Brian C. Toy & Narsing A. Rao

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  1. Youning Zhang
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  2. Brian C. Toy
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  3. Narsing A. Rao
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Correspondence to Brian C. Toy .

Editor information

Editors and Affiliations

  1. Department of Ophthalmology, Oregon Health & Science University Department of Ophthalmology, Portland, OR, USA

    Daniel Albert

  2. Massachusetts Eye and Ear, Boston, MA, USA

    Joan Miller

  3. Department of Ophthalmology, University of Illinois at Chicago Department of Ophthalmology, Chicago, IL, USA

    Dimitri Azar

  4. Department of Ophthalmology, Harvard Medical School, Boston, MA, USA

    Lucy H. Young

Section Editor information

  1. Department of Ophthalmology, Massachusetts Eye and Ear Infirmary, Boston, MA, USA

    Lucia Sobrin

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Zhang, Y., Toy, B.C., Rao, N.A. (2020). Vogt-Koyanagi-Harada Disease (Uveomeningitic Syndrome). In: Albert, D., Miller, J., Azar, D., Young, L. (eds) Albert and Jakobiec's Principles and Practice of Ophthalmology. Springer, Cham. https://doi.org/10.1007/978-3-319-90495-5_21-1

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  • DOI: https://doi.org/10.1007/978-3-319-90495-5_21-1

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