Voyt-Koyanagi-Harada syndrome

• Multisystem inflammatory disease affecting eyes, ears, brain, skin and hair
• T cell mediated autoimmune disorder against melanocyte antigens
• Higher prevalence in darker skinned races
• F>M
• 3rd and 4th decades
• Associated with HLA-DR4, especially HLA-DRB1*0405 (associated with melanocyte proteins)

Clinical features (4 phases)

• Prodromal phase: fever, meningism, auditory symptoms/tinnitus

• Anterior uveitic phase: bilateral granulomatous with iris nodules

  • Posterior uveitis: multifocal choroiditis, multifocal sensory exudative retinal detachments, choroidal depigmentation (sunset glow fundus), Dalen-Fuch’s nodules (peripheral yellow-white choroidal granulomas), subretinal fibrosis
  • Painful, photophobic, visual loss
  • This stage responds well to corticosteroids (which can tapered slowly)

• Convalescent phase: resolution of RDs, sunset glow fundus, perilimbal vitiligo (Sugiura sign), alopecia, poliosis

• Chronic recurrent phase: recurrent granulomatous uveitis, cataract, glaucoma, CNV, subretinal fibrosis

• Systemic features:

  • Cutaneous: vitiligo, alopecia, poliosis
  • Auditory: tinnitus, deafness, vertigo
  • Neurological: meningism, encephalitis, cranial neuropathies

Tests:

• OCT
• FFA: focal areas of delayed choroidal perfusion, multifocal leakage, placoid areas of hyperfluorescence, SRF pooling, optic nerve staining
• USS: choroidal thickening
• LP: lymphocytic pleocytosis

Refer to the diagnostic criteria table for VKH from the American Uveitis Society