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.