Vogt-Koyanagi-Harada Disease

Four clinical stages:

  1. Prodromal

  2. Acute

  3. Chronic convalescent

  4. Chronic recurrent

Acute stages:

  • Prodromal stage of neurologic and auditory disturbances

  • Bilateral posterior uveitis + diffuse granulomatous choroiditis

  • Vitritis

  • Optic disc oedema

  • Exudative RD – multifocal and shallow RD

  • Retinal +/- choroidal folds


FFA findings:

  • Patchy hypofluorescence (delayed choroidal filling)

  • Early frames – Multiple punctate hyperfluorescent dots at RPE.

  • Late frames – hyperfluorescent dots enlarge and stains surrounding SRF (extent of exudative RD) + optic disc hyperfluorescence.

  • Alternate hypofluorescent / hyperfluorescent bands – indicate folds.


Chronic stages:

  • Variable degree of choroidal depigmentation

  • Sunset glow fundus (loss of choroidal melanocytes)

  • Focal RPE atrophy – nummular depigmented round well-defined chorioretinal scars at mid-peripheral.

  • Hyperpigmented linear focal lesions – RPE migration/clumping.

  • Cutaneous changes – vitiligo, alopeica or poliosis of eyelashes/brows.

  • Granulomatous AAU.


Management:

  1. Systemic corticosteroids.

  2. Immunosuppressive agents

  3. OCT to monitor response to treatment – SRF / RPE undulations

  4. Treat complications – cataract, glaucoma, CNV or subretinal fibrosis accordingly.

 
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