Skip to main content

Table 2 Revised Diagnostic Criteria of Vogt-Koyanagi-Harada disease proposed by the International Nomenclature Commitee [59]

From: Vogt-Koyanagi-Harada disease: review of a rare autoimmune disease targeting antigens of melanocytes

1. No history of penetrating ocular trauma or surgery preceding the initial onset of uveitis.

2. No clinical or laboratory evidence suggestive of other ocular disease entities.

3. Bilateral ocular involvement (a or b must be met, depending on the stage of disease when the patient is examined):

 a. Early manifestations of disease:

  I. Evidence of diffuse choroiditis (with or without anterior uveitis, vitreous inflammatory reaction or optic disc hyperemia), which may manifest as (A) focal areas of subretinal fluid, or (B) bullous exudative retinal detachments.

  II. If equivocal fundus findings, then both:

   A. Fluorescein angiography showing focal delayed choroidal perfusion, multiple areas of pinpoint leakage, large placoid areas of hyperfluorescence, pooling within subretinal fluid, and optic nerve staining;

   B. Ultrasonography showing diffuse choroidal thickening without evidence of posterior scleritis.

 b. Late manifestations of disease:

  I. History suggestive of prior presence of early findings noted in 3a and either (II) or (III) below, or multiple signs from (III) below:

  II. Ocular depigmentation: either (A) sunset glow fundus or (B) Sugiura sign.

  III. Other ocular signs including (A) nummular chorioretinal depigmented scars, or (B) retinal pigment epithelium clumping and/or migration, or (C) recurrent or chronic anterior uveitis.

4. Neurological/auditory findings (may resolve by time of evaluation):

 a. Meningismus (malaise, fever, headache, nausea, abdominal pain, stiffness of the neck and back, or a combination of these factors); note that headache alone is not sufficient to meet definition of meningismus.

 b. Tinnitus

 c. Cerebrospinal fluid pleocytosis

5. Integumentary findings (not preceding onset of central nervous system or ocular disease):

 a. Alopecia, or

 b. Poliosis, or

 c. Vitiligo.

Complete VKHD: criteria 1–5 must be present

Incomplete VKHD: criteria 1–3 and either 4 or 5 must be present

Probable VKHD (isolated ocular disease): criteria 1–3 must be present