Vogt-Koyanagi-Harada Disease Clinical Presentation

  • Author: R Christopher Walton, MD; Chief Editor: Hampton Roy Sr, MD   more...
 
Updated: Apr 30, 2012
 

History

Four clinical stages have been described in VKH disease, consisting of the prodromal stage, uveitic stage, chronic stage, and recurrent stage.

  • Prodromal stage
    • The prodrome typically lasts for a few days and is characterized by fever, headache, meningismus, nausea, vertigo, orbital pain, and tinnitus. CSF pleocytosis occurs in more than 80% of patients during this stage. Photophobia and tearing may develop, and patients also may note that their skin and hair is sensitive to touch during this stage.
    • Uncommon manifestations during the prodrome include cranial nerve palsies and optic neuritis.
    • Some patients may not develop or report the symptoms characteristic of the prodrome.
  • Uveitic stage
    • The acute uveitis stage follows the prodromal stage by several days in most patients. During this stage, the most common symptom is acute bilateral blurring of vision. As many as 70% of patients present with bilateral blurring of vision, and, in the remaining patients, the fellow eye is involved within several days in most cases.
    • Clinically, this is manifest as bilateral posterior uveitis with retinal edema, optic disc hyperemia or edema, and eventually serous retinal detachments. Often, an accompanying anterior uveitis characterized by mutton-fat keratic precipitates and iris nodules are present. The intraocular pressure may be elevated because of forward rotation of the lens-iris diaphragm.
    • This stage typically lasts for several weeks.
  • Chronic stage
    • During the chronic stage, ocular and dermatologic manifestations are common. Depigmentation of the choroid begins within the first 3 months after the onset of the disease. Areas of hyperpigmentation also may develop in the fundus. Dalen-Fuchs nodules may be seen in the peripheral and midperipheral retina. These nodules are small yellow lesions that typically are located in the midperiphery of the retina. Eventually, the lesions fade and become atrophic.
    • Dermatologic changes include vitiligo and poliosis of the lashes, eyebrows, and hair. The vitiligo tends to be distributed symmetrically over the head, eyelids, and trunk.
    • The duration of the chronic stage is typically several months but may last for many years.
  • Recurrent stage
    • During the recurrent stage, patients may develop recurrent or chronic anterior uveitis. In some patients, low-grade choroidal inflammation may accompany the anterior uveitis, which may require indocyanine green angiography for visualization.[11] Recurrent posterior uveitis with serous retinal detachment is rare. Patients treated with corticosteroids and/or immunomodulator therapy for 6 months or less may be at higher risk for recurrent serous retinal detachment.[12]
    • Ocular complications are relatively common during this stage and include cataracts, glaucoma, choroidal neovascularization, and subretinal fibrosis.
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Physical

Patients suspected of having VKH disease should undergo a thorough physical examination to search for cutaneous, neurologic, and ophthalmic manifestations of the disorder.

  • Cutaneous manifestations
    • Sensitivity to touch of the hair and skin may be noted during the prodromal stage.
    • Vitiligo, poliosis, and alopecia typically develop during the chronic stage. Vitiligo often is distributed symmetrically over the head, face, and trunk. The sacral region is a common site for the development of vitiligo.
    • Poliosis may involve the scalp hair, eyebrows, and eyelashes.
  • Neurologic manifestations
    • Meningeal signs develop during the prodromal stage and include meningismus, headache, and occasional confusion. CSF pleocytosis is relatively common during the prodrome.
    • Focal neurologic signs include cranial nerve palsies, hemiparesis, transverse myelitis, and ciliary ganglionitis.
    • Inner ear disorders, including dysacusis, tinnitus, and vertigo, occur in as many as 75% of patients. Cochlear hearing loss occurs mainly in high-frequency ranges. Inner ear dysfunction improves several months after onset in most patients.
  • Ophthalmic manifestations
    • Visual acuity may be decreased markedly in both eyes at the onset of the uveitic stage. Patients may present with unilateral loss of vision, but most develop bilateral disease within the first 10 days following onset.
    • Ocular adnexa involvement includes poliosis of the scalp, eyebrows, or eyelashes, which may develop during the convalescent stage of VKH disease. Vitiligo also may occur on the eyelids and face during this stage.
    • Anterior segment
      • Perilimbal vitiligo (Sugiura sign) is one of the earliest manifestations of depigmentation but is uncommon, except in Japanese patients.
      • A granulomatous or nongranulomatous anterior uveitis may occur. Busacca nodules, Koeppe nodules, and mutton-fat keratic precipitates are characteristic of granulomatous anterior uveitis.
      • Posterior synechiae may be noted, especially in chronic cases. Pupillary membrane formation is relatively common.
      • Some patients may present with a shallow anterior chamber due to edema and infiltration of the ciliary body, resulting in forward rotation of the lens-iris diaphragm and possible angle-closure glaucoma.
      • Cataracts may develop as a result of chronic inflammation and/or chronic corticosteroid therapy.
      • Glaucoma may occur secondary to pupillary block or angle closure or in association with chronic uveitis.
    • Posterior segment
      • Anterior vitreous cells may be noted, especially in patients with severe anterior uveitis.
      • Optic disc hyperemia or edema may be present.
      • One of the earliest retinal manifestations is retinal edema, which is often located within the posterior pole. This typically is followed by the development of bilateral multifocal serous retinal detachments. The detachments occur most commonly in the inferior retina.
      • During the chronic stage of the disease, the serous detachments resolve and retinal pigment epithelium (RPE) alterations are common, including depigmentation, demarcation lines, and areas of hyperpigmentation. The fundus of Asian and Hispanic patients may develop the characteristic red-orange appearance of the sunset-glow fundus, although this is relatively uncommon in other groups of patients. Areas of hyperpigmentation are also common and reflect changes occurring at the level of the RPE. Subretinal fibrosis, RPE migration, and disciform scars also may occur.
      • Neovascularization of the disc and retina may develop and can result in vitreous hemorrhage.
      • Choroidal neovascularization of the macula may occur in the chronic stage and can result in profound loss of visual acuity.Bilateral multifocal serous detachments in a patieBilateral multifocal serous detachments in a patient with Vogt-Koyanagi-Harada disease. Disc hyperemia is evident in the right eye.
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Causes

The pathogenesis of VKH disease is uncertain. See Pathophysiology.

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Contributor Information and Disclosures
Author

R Christopher Walton, MD  Professor, Director of Uveitis and Ocular Inflammatory Disease Service, Department of Ophthalmology, University of Tennessee College of Medicine

R Christopher Walton, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Healthcare Executives, American Uveitis Society, Association for Research in Vision and Ophthalmology, and Retina Society

Disclosure: Nothing to disclose.

Specialty Editor Board

John D Sheppard Jr, MD, MMSc  Professor of Ophthalmology, Microbiology and Molecular Biology, Clinical Director, Thomas R Lee Center for Ocular Pharmacology, Ophthalmology Residency Research Program Director, Eastern Virginia Medical School; President, Virginia Eye Consultants

John D Sheppard Jr, MD, MMSc is a member of the following medical societies: American Academy of Ophthalmology, American Society for Microbiology, American Society of Cataract and Refractive Surgery, American Uveitis Society, and Association for Research in Vision and Ophthalmology

Disclosure: Nothing to disclose.

Francisco Talavera, PharmD, PhD  Adjunct Assistant Professor, University of Nebraska Medical Center College of Pharmacy; Editor-in-Chief, Medscape Drug Reference

Disclosure: Medscape Salary Employment

Steve Charles, MD  Director of Charles Retina Institute; Clinical Professor, Department of Ophthalmology, University of Tennessee College of Medicine; Adjunct Professor of Ophthalmology, Columbia College of Physicians and Surgeons; Clinical Professor Ophthalmology, Chinese University of Hong Kong

Steve Charles, MD is a member of the following medical societies: American Academy of Ophthalmology, American Society of Retina Specialists, Club Jules Gonin, Macula Society, and Retina Society

Disclosure: Alcon Laboratories Consulting fee Consulting; OptiMedica Ownership interest Other; Topcon Medical Lasers Consulting fee Consulting

Lance L Brown, OD, MD  Ophthalmologist, Affiliated With Freeman Hospital and St John's Hospital, Regional Eye Center, Joplin, Missouri

Disclosure: Nothing to disclose.

Chief Editor

Hampton Roy Sr, MD  Associate Clinical Professor, Department of Ophthalmology, University of Arkansas for Medical Sciences

Hampton Roy Sr, MD is a member of the following medical societies: American Academy of Ophthalmology, American College of Surgeons, and Pan-American Association of Ophthalmology

Disclosure: Nothing to disclose.

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Bilateral multifocal serous detachments in a patient with Vogt-Koyanagi-Harada disease. Disc hyperemia is evident in the right eye.
Fluorescein angiography of the left eye in a patient with Vogt-Koyanagi-Harada disease. Mid phase is shown on the left with multiple areas of hyperfluorescence at the level of the retinal pigment epithelium. Late phase of the same angiogram (right) reveals multiple placoid areas of hyperfluorescence at the level of the retinal pigment epithelium and pooling of dye in the areas of serous detachment.
Patient with progressive dysacusis and recent onset of visual loss. Fundus photo shows a large multifocal serous detachment of the right eye. B-scan ultrasonography reveals posterior choroidal thickening with an overlying retinal detachment.
Patient with progressive dysacusis and recent onset of visual loss is shown here following 6 weeks of systemic corticosteroid therapy. Diffuse depigmentation of the choroid with retinal pigment epithelium migration is seen. Residual retinal striae are present in the peripapillary region. B-scan ultrasonography shows resolution of retinal detachment and choroidal thickening.
 
 
 
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