2. Vogt-Koyanagi-Harada (VKH) disease is a multisystemic
disorder characterized by granulomatous panuveitis with
exudative retinal detachments that is often associated
with neurologic and cutaneous manifestations.
3. Commonly affects darkly pigmented ethnic groups
Uncommon among whites
Women more commonly affected than Men Except in Japanese
populations
Most common in second to fouth decade of life
4. Unknown
Experimental evidence suggests Cell mediated autoimmune
process against Melanocytes of all organ systems(genetically
susceptible individuals)
T helper-1 cells & upregulation of associated cytokines(IL-2,IL-6
&INF-gamma) also plays a role
Recenty study suggests that IL-23(differentiation of IL-17
producing CD4 helper T lymphocytes) responsible for development
& maintanence of autoimmune process
5. Sensitisation to melanocyte antigenic peptides by cutaneous
injury/viral infections-possible trigger
Tyrosinase/Tyrosinase related protiens(75 Kda protein & S-100
protein targets melaocytes
6. HLA-DR4 in Japanese population
HLA DRB1 *0405,HLA DRB1*0410 haplotypes-stongly associated
risk
84% Hispanic patients from Southern California found to have high
relative risk with HLA-DR1 than HLADR4
7. Diffuse, nonnecrotizing, granulomatous inflammation of the uvea
Acute uveitic stage characterized by exudative detachment of the
neurosensory retina
An inflammatory infiltrate is noted at the level of the pigment
epithelium, and these focal disruptions of the RPE produce the
characteristic focal leakage noted on fluorescein angiography
The choriocapillaris is preserved in the acute uveitic stage, but
becomes involved during the chronic recurrent stage of the disease
Choroidal melanocytes are damaged by the inflammatory process;
loss of these melanocytes clinically manifests as a sunset glow fundus
8. Flu like symptoms – 3-5 days
Headache,neck stiffness,confusion ,nausea,fever,
dysacusia,tinnitus,orbital pain,photophobia Hypersensitivity of
skin & hair
Focal Neurological signs:Cranial
neuropathies,Hemiparesis,Aphasia,Transverse myelitis &
ganglionitis
CSF Analysis:lymphocytic pleocytosis,Normal level of glucose>80%
of patients(may persist up to 8 wks)
9. Sequential blurring of vision in both eyes 1-2 days after the onset
of CNS signs
Granulamatous anterior uveitis
Variable degree of vitritis
Thickening of posterior choroid with elevation of peripapillary
retinal choroidal layer
Hyperemia & edema of optic disc
Multiple serous retinal detachments
11. Multiple, focal areas of
leakage are noted at the
level of the RPE, and
subretinal fluid
accumulation may be
seen
Right eye, early
arteriovenous phase of
fluorescein angiogram
exhibiting multifocal
areas of
hyperfluorescence at the
level of the RPE along
with pooling of dye in
the subretinal space
12. (a) Multiple serous retinal detachments in the acute
uveitic stage of VKH. Also note marked optic disk
hyperemia.
(b) OCT of the same eye confirms exudative retinal
detachment as well as retinal edema.
13.
14. Focal serous RD often shallow(clover leaf pattern)
coalasce to form large bullous exudative RD-profound visual
loss
Less commonly,mutton fat KP’s,iris nodules at pupillary
margin are observed
AC may be shallow due to forward displacement of lens-iris
diaphragm(ciliary body edema & annular choroidal
detachment)
IOP may be elevated or low secondary to ciliary body shut
down
15. Several weeks later to several months
Cutaneous symptoms
Resolution of exudative RD
Gradual depigmentation of choroid leads to classic
orange-red discolouration(Sunset glow fundus)
In addition,small,round discrete depigmented lesions
–inferior mid peripheral fundus (nummular scars )
Juxta papillary depigmentation (pale disc) may also
occur
Perilimbal vitiligo(Sugiura sign)-85% of japanese
patients,not in whites
16.
17.
18. Integumentary changes:Vitiligo,poliosis,alopecia corresponds to
fundus depigmentation occurs in 30% of patients
Skin & hair changes usually occur weeks – months after onset of
ocular inflamation but it may occur simultaneously
10-63% develops vitiligo on ethnic background
19. A smoldering panuveitis with acute episodic exacerbations of
granulomatous anterior uveitis
Iris nodules may appear as round, whitish, well-circumscribed
lesions on a background of atrophic iris stroma
Posterior segment recurrences associated with
vitritis,papillitis,multifocal choroiditis,exudative RD- rare
Anterior segment recurrence coincides with subclinical choroidal
inflammation requires systemic therapy
Sequelae of chronic inflammation leads to
PSCC,glaucoma,CNV,sub retinal fibrosis
20.
21. 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 (early n late - must be met,
depending on the stage of disease when the patient is
examined)
4. Neurological/auditory findings (may resolve by time of
evaluation)
5. Intergumentory finding (not preceding onset of central
nevous system or ocular disease)
24. Usually clinical
Characterised by Exudative RD in acute stage,Sunset glow fundus
in chronic recurrent stage
CBC,Mantoux test,TPHA-To rule out infectious cause
FFA,ICG Angiography,OCT,USG,Lumbar puncture help in
confirming diagnisis
25. Acute uveitic stage:
Numerous hyperfluorescent foci at level of RPE in early stage
followed by pooling of dye in sub-retinal space in areas of
Neurosensory dtachment
Majority shows disc leakage,CME & retinal vascular leakage are
uncommon
Convalescent & Chronic recurrent stage:
Focal RPE loss and atrophy produce multiple hyperfluorescent
window defects without progressive staining
26.
27. Highlights choroidal pathology
Shows delay in choriocapillaries & choroidal vessel perfusion
Early choroidal vessel stromal hyperfluorescence & leakage
Disc hyperfluorescence
Multiple hypofluorescent spots throughout the fundus indicates
foci of lymphocytic infiltration
Hyperfluorescent pinpoint changes with in areas of exudative RD
Hypofluorescent spots-sensitive marker and follow up of sub
clinical choroidal inflammation(when fundoscopic & FFA findings
are unremarkable)
28. ICG photographs of the right eye of a patient in the acute phase of VKHD
showing (A) patchy hypofluorescence during the early angiographic phase,
(B) large choroidal stromal vessel hyperfluorescence with fuzzy choroidal
vessels in the early phase, (C) hypofluorescent dark dots during the
intermediate phase of angiography, and (D) diffuse choroidal
hyperfluorescence in the late phase.
29. Helpful in diagnosis in presence of media opacity
Shows diffuse,low to medium reflective thickening of posterior
choroid ,most prominent in peripapillary area with extension to
equatorial region
Exudative RD
Vitreous opacification
Posterior thickening of sclera
30.
31. Helps in diagnosis &
monitoring of
Serous macular
detachment
CME
CNVM
32. Done in atypical cases who presented early with neurological signs
Shows lymphocytic pleocytosis
34. Sympathetic Ophthalmia Vogt-Koyanagi-Harada Syndrome
Age All ages 20-50 years of age
Racial predisposition None Asian and Black
Penetrating trauma Almost always present Absent
Skin changes Uncommon or unrelated Common (60-90%)
CNS findings Uncommon Common (85%)
Hearing dysfunction Uncommon Common (75%)
Retinal serous
detachment
Uncommon Frequently seen
Choriocapillaris
involvement
Usually absent Frequently seen
CSF findings Usually normal Pleocytosis (84%)
36. Immunosupprasants
Cyclosporine, 5 mg/kg/ day
Tacrolimus 0.1–0.15 mg /kg /day
Cytotoxic Agents[10,42]
Azathioprine, 1–2.5 mg/kg/ day
Mycophenylate mofetil, 1-3 g/day
Cyclophosphamide 1–2 mg/ kg/ day
Chlorambucil 0.1 mg/kg/day with adjustment of dose every 3
weeks up to a maximum of 18 mg/day
37. Good with prompt and agressive therapy
In addition to cataract and glaucoma,subretinal fibrosis and
choroidal neovascular membrances may occur
38. Albert and jacobie 3rd edition vol 1.page chapter 97
Uveitis fundamentals and clinical practice – robert &
scott:4th edition page no 303 -315