Vogt-Koyanagi-Harada disease (VKHD) is a bilateral, chronic granulomatous panuveitis associated with central nervous system, auditory, and integumentary manifestations.
Classically, the disease begins with a prodromal phase of neuroauditory symptoms, followed by an acute uveitis phase, and finally, the chronic stage manifestations.
Revised Diagnostic Criteria of Vogt-Koyanagi-Harada Disease (VKHD)
The diagnosis of VKHD is primarily based on clinical features. The American Uveitis Society (AUS) adopted the following diagnostic criteria:
- No history of ocular trauma and/or surgery;
- At least three of the following four signs:
- Bilateral chronic iridocyclitis;
- Posterior uveitis (multifocal exudative retinal or retinal pigment epithelium (RPE) detachments; disc hyperemia or edema; or “sunset glow fundus”, which is a yellow-orange appearance of the fundus due to depigmentation of the RPE and choroid);
- Neurologic signs (tinnitus, neck stiffness, cranial nerve or central nervous system symptoms or cerebral spinal fluid pleocytosis);
- Cutaneous findings (alopecia, poliosis or vitiligo).
Revised Diagnostic Criteria of Vogt-Koyanagi-Harada disease proposed by the International Nomenclature Commitee
- No history of penetrating ocular trauma or surgery preceding the initial onset of uveitis.
- No clinical or laboratory evidence suggestive of other ocular disease entities.
- Bilateral ocular involvement (a or b must be met, depending on the stage of disease when the patient is examined):
- Early manifestations of disease:
- 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.
- If equivocal fundus findings, then both:
- 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;
- Ultrasonography showing diffuse choroidal thickening without evidence of posterior scleritis.
- Late manifestations of disease:
- History suggestive of prior presence of early findings noted in 3a and either (II) or (III) below, or multiple signs from (III) below:
- Ocular depigmentation: either (A) sunset glow fundus or (B) Sugiura sign (perilimbal vitiligo).
- 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.
- Early manifestations of disease:
- Neurological/auditory findings (may resolve by time of evaluation):
- 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.
- Cerebrospinal fluid pleocytosis
- Integumentary findings (not preceding onset of central nervous system or ocular disease):
- Alopecia, or
- Poliosis, or
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
- Burkholder BM. Vogt-Koyanagi-Harada disease. Curr Opin Ophthalmol. 2015 Nov;26(6):506-11. [Medline]
- Lavezzo MM, Sakata VM, Morita C, Rodriguez EE, Abdallah SF, da Silva FT, Hirata CE, Yamamoto JH. Vogt-Koyanagi-Harada disease: review of a rare autoimmune disease targeting antigens of melanocytes. Orphanet J Rare Dis. 2016 Mar 24;11:29. [Medline]
- O’Keefe GA, Rao NA. Vogt-Koyanagi-Harada disease. Surv Ophthalmol. 2017 Jan – Feb;62(1):1-25. [Medline]
Created Oct 02, 2019.