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Table 3 Diagnostic criteria for chronic recurrent Vogt-Koyanagi-Harada disease

From: Vogt-Koyanagi-Harada disease: the step-by-step approach to a better understanding of clinicopathology, immunopathology, diagnosis, and management: a brief review

1. No ocular trauma or surgery preceding onset of diseasea
2. Bilateral involvement (verified with ICGA and/or EDI-OCT)a
3. Exclusion of other infectious, inflammatory or masquerading entities, in particular other stromal choroiditis entities (i.e. tuberculosis, sarcoidosis or syphilis)a
4. Signs and symptoms of more than 4 weeks´ duration before treatment initiationa
5. Ocular depigmentation findings (i.e., sunset glow fundus, Sugiura sign, Dallen-Fuchs-like nodules)
6. Integumentary findings (i.e., vitiligo, poliosis)
7. Choroidal atrophy (evidenced by thinning and/or distortion of vascular architecture on EDI-OCT and/or ICG)
8. Treatment refractoriness, defined as absence of inflammatory improvement or persistence of inflammation (namely not achieving a two-step decrease in the level of inflammation or a decrease to grade 0 and/or persistence of retinal detachment), despite a minimum of 4 to 6 weeks of systemic therapy [105]
9. Presence of ocular complications, such as cataract, glaucoma, hypotony, choroidal neovascularization.
10. Presence of granulomatous anterior segment inflammation (namely Koeppe/Bussacca iris nodules plus mutton-fat keratic precipitates) [6, 44].
  1. aEssential criteria