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20 year old Hispanic woman with bilateral blurry vision, nausea, weakness and severe headaches
Digital Journal of Ophthalmology 2005
Volume 11, Number 11
July 10, 2005
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Szilard Kiss | Massachusetts Eye and Ear Infirmary
Diagnosis and Discussion
Vogt-Koyanagi-Harada disease

Vogt-Koyanagi-Harada disease (VKH) is a systemic autoimmune disorder directed against antigens most likely associated with melanocytes present in such tissues as the choroid, the meninges, the inner ear, and the skin. It is characterized by bilateral, chronic, diffuse granulomatous uveitis, accompanied by characteristic neurological, auditory and integumentary features. The eye is typically the most involved organ, and visual sequelae are the most frequent and debilitating consequences of the disease.

Patients are typically 20 to 50 years old and have no history of either surgical or accidental ocular trauma. VKH affects mainly darkly pigmented races, such as East and Southeastern Asians, Asian Indians, Middle Easterners, Hispanics, and Native Americans; it is uncommon in Caucasians. VKH is also rare in Africans, suggesting that skin pigmentation alone is not the sole etiologic factor in its pathogenesis.

Based on revised diagnostic criteria, the disease is classified as complete, incomplete or probable based on the presence of extraocular findings (neurological, auditory and integumentary). No matter the form of the disease the patient manifests, three conditions must be present for the diagnosis of VKH: (1) no previous history of penetrating ocular trauma (either surgical or accidental); (2) no clinical or laboratory evidence suggestive of other ocular disease; and (3) bilateral ocular involvement.

The clinical course of VKH is divided in four phases: prodromal (mimics a viral infection), uveitic (bilateral diffuse uveitis with papillitis and exudative retinal detachment), convalescent (tissue depigmentation), and chronic recurrent (recurrent uveitis and ocular complications).

The pathogenesis of VKH is thought to be related to an aberrant T cell-mediated immune response directed against self-antigens found on melanocytes. VKH has been linked to human leukocyte antigen DR4 (HLA-DR4) and HLA-Dw53, with strongest associated risk for HLA-DRB1*0405 haplotype.

Comprehensive diagnostic criteria for VKH were revised during the 2001 First International Workshop on Vogt-Koyanagi-Harada Disease. The diagnosis of VKH is clinical, and differential includes sympathetic ophthalmia, sarcoidosis, primary intraocular B-cell lymphoma, posterior scleritis, and uveal effusion syndrome.

The standard initial therapy for VKH is prompt and aggressive use of systemic corticosteroids, to which the disease is especially responsive, particularly in the early stages. Typical dosing regimens range from 1.0 to 2.0 mg/kg/day of oral prednisone or 200 mg of intravenous methylprednisolone for 3 days, followed by or high-dose corticosteroids. Early therapy with a slow taper based on clinical response, usually over a minimum of 6 months, has been shown to improve the prognosis by reducing the length of disease, increasing the incidence of a convalescent phase, and decreasing the extraocular manifestations. Other immunomondulaory agents (most oftentimes cyclosporine) may be needed for non-responsive patients or when corticosteroid side-effects are not tolerated. Visual prognosis with VKH is generally good with prompt diagnosis and aggressive immunomodulatory treatment.