Vogt-Koyanagi-Harada Disease

Four clinical stages:

1. Prodromal

2. Acute

3. Chronic convalescent

4. Chronic recurrent

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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.