Thursday, April 10, 2008

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

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