Vogt-Koyanagi-Harada syndrome

Abstract

The Vogt-Koyanagi-Harada syndrome (VKH) is a bilateral, diffuse granulomatous uveitis associated with poliosis, vitiligo, alopecia, and central nervous system and auditory signs. These manifestations are variable and race dependent. This inflammatory syndrome is probably the result of an autoimmune mechanism, influenced by genetic factors, and appears to be directed against melanocytes. On histopathologic examination typical cases show nonnecrotizing diffuse granulomatous panuveitis with initial sparing and late involvement of the choriocapillaris and formation of Dalen-Fuchs' nodules. Fluorescein angiography, lumbar puncture, and echography are useful adjuncts in the diagnosis and management of VKH syndrome. Patients with this syndrome are treated generally with high dose systemic corticosteroids or, when necessary, with cyclosporine or cytotoxic agents. The prognosis of patients with VKH syndrome is fair, with nearly 60% of patients retaining vision of 20/30 or better. The complications of VKH syndrome that lead to visual loss include cataracts in about 25% of patients, glaucoma in 33%, and subretinal neovascular membranes (SRNVMs) in about 10%; the latter, however, are an important cause of late visual loss. These complications usually require medical and/or surgical intervention, including photocoagulation. The major risk factor for the development of cataracts, SRNVMs, and, to some extent, glaucoma, is chronic recurrent intraocular inflammation that may be resistant to corticosteroid therapy. It appears that initial treatment with high dose corticosteroids, combined with prolonged corticosteroid therapy at appropriate dosage, may minimize these complications and may improve visual prognosis.