Vogt-Koyanagi-Harada disease is a multisystem disorder characterized by granulomatous panuveitis with exudative retinal detachments that are often associated with neurologic and cutaneous manifestations.
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Epidemiology
Vogt-Koyanagi-Harada disease usually affects those of Asian, Middle Eastern, Asian Indian, Native American or Hispanic ethnicity 5. Patients are often typically middle-aged (range of 20-50 years old) at presentation 7.
Diagnosis
The revised diagnostic criteria for Vogt-Koyanagi-Harada disease were published in 2001 9:
Complete Vogt-Koyanagi-Harada disease
Criteria 1 to 5 must be present:
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
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bilateral ocular involvement (a or b must be met, depending on the stage of disease when the patient is examined)
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a: early manifestations of disease
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evidence of a diffuse choroiditis (with or without anterior uveitis, vitreous inflammatory reaction, or optic disk hyperemia), which may manifest as one of the following:
focal areas of subretinal fluid, or
bullous serous retinal detachments
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with equivocal fundus findings, both of the following must be present as well:
focal areas of delay in choroidal perfusion, multifocal areas of pinpoint leakage, large placoid areas of hyperfluorescence, pooling within subretinal fluid, and optic nerve staining (listed in order of sequential appearance) by fluorescein angiography, and
diffuse choroidal thickening, without evidence of posterior scleritis by ultrasonography
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b: late manifestations of disease.
history suggestive of prior presence of findings from 3a, and either both (2) and (3) below, or multiple signs from (3)
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ocular depigmentation (either of the following manifestations is sufficient):
sunset glow fundus, or
Sugiura sign (i.e. perilimbal vitiligo)
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other ocular signs:
nummular chorioretinal depigmented scars, or
retinal pigment epithelium clumping and/or migration, or
recurrent or chronic anterior uveitis
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neurological/auditory findings (may have resolved by time of examination)
meningismus (malaise, fever, headache, nausea, abdominal pain, stiffness of the neck and back, or a combination of these factors, but headache alone is insufficient), or
tinnitus, or
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integumentary finding (not preceding onset of central nervous system or ocular disease)
alopecia, or
poliosis, or
vitiligo
Incomplete Vogt-Koyanagi-Harada disease
Criteria 1 to 3 and either 4 or 5 must be present:
no history of penetrating ocular trauma or surgery preceding the initial onset of uveitis, and
no clinical or laboratory evidence suggestive of other ocular disease entities, and
bilateral ocular involvement
neurologic/auditory findings; as defined for complete Vogt-Koyanagi-Harada disease above, or
integumentary findings; as defined for complete Vogt-Koyanagi-Harada disease above
Probable Vogt-Koyanagi-Harada disease
Criteria 1 to 3 must be present (isolated ocular disease):
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 as defined for complete Vogt-Koyanagi-Harada disease above
Clinical presentation
Vogt-Koyanagi-Harada disease typically presents in stages 11:
prodromal stage: flu-like presentation, including meningism and vestibulo-auditory symptoms
uveitic stage: bilateral posterior uveitis progressing to panuveitis
convalescent stage: evidence of depigmentation, including vitiligo, poliosis, choroidal depigmentation, and alopecia
chronic-recurrent stage: chronic sequela of posterior uveitis and acute exacerbations of anterior uveitis, including risk of permanent blindness
Pathology
Vogt-Koyanagi-Harada disease is considered an inflammatory disease occurring probably as a result of an autoimmune mechanism, influenced by genetic factors, and appears to be directed against antigenic targets on melanocytes 11.
Radiographic features
Ultrasound
B-mode ocular ultrasound features of Vogt-Koyanagi-Harada disease are:
low to medium reflective thickening of the posterior choroid
mild thickening of the sclera and/or episclera adjacent to areas of choroidal thickening
vitreous opacities
subretinal septations may be seen
MRI
MR imaging may detect early CNS involvement by Vogt-Koyanagi-Harada disease before the onset of neurologic symptoms which include:
typical bilaterality of ocular findings
scattered periventricular white matter lesions on T2-weighted imaging/FLAIR
pachymeningeal enhancement
bilateral contrast enhancement of the choroid is seen along diffuse choroidal thickening with scleral sparing and retinal detachment
Treatment and prognosis
First-line treatment is with prompt systemic corticosteroid therapy 10, however, steroid-sparing immunomodulators (or immunosuppressants) have also proven to be effective treatments 12. Furthermore, steroid-sparing immunomodulators are the treatment of choice for the long-term prevention of recurrence 13.
History and etymology
What is now known as Vogt-Koyanagi-Harada disease was initially described as three separate similar entities by 11:
Alfred Vogt (1879-1943), Swiss ophthalmologist, in 1906
Einosuke Harada (1892-1946), Japanese ophthalmologist, in 1926
Yoshizo Koyanagi (1880-1954), Japanese ophthalmologist, in 1929