HIDAYAT

I11109084
Vogt Koyanagi Harada Syndrome
Introduction
Vogt-Koyanagi-Harada (VKH) syndrome is a
systemic autoimmune disease in which the main
target is melanin-containing-cells present in the
eye,meninges, ear and skin
Pathogenesis

Pathology and pathogenesis
diffuse thickening of the uveal tract -> non-
necrotizing granulomatous inflammation
Inflammatory cell infiltration, consisting of
lymphocytes and focal aggregates of epithelioid
histiocytes and multinucleated giant cells, spares
the inner layer of the choroid (choriocapillaris)
and the overlying retina
The focal collection of lymphocytes, pigment-
laden macrophages, epithelioid cells and
proliferated retinal pigment epithelium (RPE) cells
-> Daln-Fuchs nodules.
retinal detachment with collection of subretinal
fluid most likely results from alterations in the
RPE as upstream effect of choroidal
compromise.
The inflammatory cell infiltration may often extend
to ciliary body and iris stroma.
The convalescent phase of the disease is
characterized by a mild to moderate non-
granulomatous inflammation with uveal infiltration
of lymphocytes, few plasma cells, and occasional
macrophages.
The choroid is depigmented -> damage of the
choroidal melanocytes -> sunset-glow fundus
numerous peripheral choroidal depigmented
small atrophic lesions involving the overlying
choriocapillaris, the RPE. These atrophic lesions
correspond to focal RPE loss with chorioretinal
adhesions
In the chronic recurrent stage, a diffuse uveal
infiltration consisting of a granulomatous process, less
prominent than in the acute stage. Chorioretinal
adhesions, with atrophy and/or proliferation of RPE
are common.
The RPE proliferation has the clinical appearance of
hyperpigmented changes on ophthalmoscopic
examination and formation of subretinal neovascular
membranes.
hyperplastic RPE, devoid of pigmentation, may be
clinically reorganized as subretinal fibrosis.
In association with the RPE changes, photoreceptor
degeneration and gliosis may be observed in the
overlying neural retina.
Clinical stage
The clinical course of VKH disease follows four
stages or phases:
prodromic, uveitic, chronic and recurrent
Prodromal stage
The prodrome typically lasts for a few days
Fever, Headache, Meningismus, Nausea, Vertigo,
Orbital pain, Tinnitus, CSF pleocytosis,
Photophobia, and optic neuritis.

Uveitic stage
acute bilateral blurring of vision
bilateral posterior uveitis with retinal edema, optic
disc hyperemia or edema, and, serous retinal
detachments.
anterior uveitis characterized by mutton-fat keratic
precipitates and iris nodules. The intraocular
pressure may be elevated.

Chronic stage
ocular and dermatologic manifestations
Occular
Depigmentation of the choroid, Dalen-Fuchs
nodules
Dermatologic
vitiligo and poliosis of the lashes, eyebrows, and
hair.

Recurrent stage
recurrent or chronic anterior uveitis, low-grade
choroidal inflammation,Recurrent posterior uveitis
with serous retinal detachment.
Retinal detachment and disc
edem


Mutton fat keratitic presipitate
Iris
nodule
polyosis
Sunset glow fundus
Complications
cataract (1042%)
Glaucoma (645%)
subretinal fibrosis (840%)
neovascular membranes (914%)
Complementary ophthalmic
examinations
Fluorescein angiography (FA)
ocular ultrasound
optic coherence tomography (OCT)
Fluorescein Angiography
Acute VKH disease
Multiple pinpoint areas of leakage at the level of
the retinal pigment epithelium (RPE) overlying
areas of choroiditis are visible during the
arteriovenous phase.
Peripapillary pinpoint hyperfluorescence, radial
folds of the choroid may be visible as alternating
dark and light bands of fluorescence.
Multiple serous retinal detachments with pooling
of dye. Other, less common findings include
retinal vascular leakage and optic disc staining.

Pinpoint area

Pooling of dye
Recovery phase of VKH disease (after treatment
with systemic corticosteroids)
Most of the acute phase abnormalities, including
exudative retinal detachment and disc edema,
resolve during this period. Fluorescein
angiography may show persistent pinpoint areas
of leakage and disc staining. Some patients may
exhibit window defects and areas of mottled
background hyperfluorescence.
Chronic VKH disease
depigmentation of the choroid, RPE atrophy are
visible, such as a moth-eaten appearance,
multiple window defects, and areas of alternating
hyperfluorescence and hypofluorescence.
Additional findings include choroidal
neovascularization, retinochoroidal and
arteriovenous anastomoses, and
neovascularization of the disc. Macular edema is
rare in this disorder but may be seen in the
chronic phase

FA
Midphase is shown on the left, with multiple areas of
hyperfluorescence at the level of the retinal pigment
epithelium (RPE).
Late phase on the same angiogram (right) reveals multiple
placoid areas of hyperfluorescence at the level of the RPE
and pooling of dye in the areas of serous detachment.
Ultrasonography
The most characteristic ultrasonographic finding
is diffuse, low to medium reflective thickening of
the posterior choroid. Additional findings include
serous retinal detachments, mild thickening of the
sclera and/or episclera adjacent to areas of
choroidal thickening, and mild vitreous opacities.
These ultrasonographic features may be useful in
monitoring the patients response to therapy.
Patient with progressive dysacusis and recent onset of
visual loss. Fundus photo shows a large, multifocal
serous detachment of the right eye. B-scan
ultrasonography reveals posterior choroidal thickening
with an overlying retinal detachment

OCT scanning
Serous retinal detachments with subretinal septa
may be visible, especially early in the disease.
Optical coherence tomography (OCT) scanning
may be useful for monitoring serous detachments
and response to therapy. Enhanced depth
imaging OCT scanning has revealed a markedly
thickened choroid in patients with active VKH
optic coherence tomography (OCT) with elevated
retinal detachment and subretinal membranes
Diagnosis
Complete disease (1 to 5 must be present)
1. No history of penetrarting ocular trauma or
surgery preceeding 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)
a) Early manifestations
Diffuse choroiditis (focal areas of subretinal fluid,
bullous serous retinal detachments )
OR, charateristics fluorescein angiography findings
(focal delayed choroidal perfusion, pinpoint
leakage, pooling within subretinal fluid, AND
echography evidence of diffuse chroidal thickening
b) Late manifestations
History suggestive of prior uveitis with the above
described characteristics AND ocular
depigmentation (sunset of glow fundus, sugiura
sign)
AND other ocular signs (numular chorioretinal
depigmented scars, retinal pigment epitelium
clumping and/or migration, or recurrent or chronic
anterior uveitis)


4. Neurological/auditory findings
Meningismus, OR tinnitus, OR cerebrospinal fluid
pleocytosis
5. Integumentary findings
Alopecia, or poliosis or vitiligo
Incomplete disease (1 to 3 and either 4 or 5
must be present)
Probable disease (isolated ocular disease; 1 to
3 must be present)

Treatment
Corticosteroids should initially be given in high
doses, i.e. 12 mg/kg/day of oral prednisone or 1
g/day of intravenous methylprednisolone for 3
days, followed by oral prednisone (1.0 mg/kg/day)
with slow tapering along 6 months or more.
Steroid is given for 6-9 months prior to tappering
off
Tappering off done by lowering the dose of
prednisone 10 mg/day weekly until the dose
reached 40 mg/day,
followed by reduced 5 mg/day dose weekly to 20
mg/day
then 2.5 mg/day weekly until the dose reached 10
mg/day
Next tappering off done by lowering the dose of
2.5 mg/day per month.
Therapy was performed at least as long as six
months

Cyclosporine A
The recommended dosage of cyclosporine is 3 to
5 mg/kg/day
Sustained control of inflammation is observed in
approximately 52% of patients within 12 months

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