Vogt-Koyanagi-Haraa (VKH) syndrome

• bilateral granulomatous panuveitis associated with serous retinal detachments, optic disc edema, neurologic abnormalities and skin pigment changes
• systemic manifestations: tinnitus, vitiligo, alopecia, headache and meningismus
• T cell-mediated autoimmune process directed against melanocyte antigens
• more prevalent in Asians, Latinos and American Indians
• women slightly > men
• any age but usually 4th-6h decades of life

4 phases

• prodromal
• acute uveitic (poor VA, severe AC inflammation w/ or w/o posterior synchiae)
• convalescent
• chronic recurrent (RPE alterations, widespread loss of choroidal melanocytes producing a sunset-glow fundus and choroidal Dalen-Fuchs-like nodules, cutaneous vitiligo, poliosis and alopecia)

Complications

• catarct, glaucoma, CNV, subretinal fibrosis, ERM, macular atrophy

Treatment

• prompt initiation of high-dose systemic corticosteroid therapy (1 to 1.5 mg/kg/day) concurrent with a corticosteroid-sparing immunosuppresive agent) , tapering patients off within 2-3 months
• rapid and aggressive treatment is important to minimize disease duration and lessen the risk of progression into a chronic recurrent form of disease and reduce the incidence of systemic and ocular complications