presentation made during uveitis session.

1. Vogt-Koyanagi-Harada Disease
Bipin Bista
Resident
Ophthalmology
NMCTH

2.  Bilateral uveitis featuring exudative retinal
detachment, usually associated with meningiomas
with or without other extraocular manifestations.
Definition

3.  An autoimmune reaction to melanocytes or to their
tyrosinase-related peptides .
Pathogenesis

4. 1. Prodromal phase
2. Uveitic phase
3. Chronic & recurrent phase
Ocular manifestations

5.  Influenza like symptoms : fever, malaise, headache,
nausea
 Blurring of vision, photophobia, redness & ocular pain
after 1-2 days after the symptoms
 Sensitivity of scalp hair to touch.
Prodromal phase

6. Uveitic phase
 Cells are present in anterior
chamber, vitreous or both.
Bilateral exudative retinal
detachment
 RD shows discrete and
shallow elevation of the
neural retina
 Cloverleaf pattern –
posterior fundus.

7. Chronic & recurrent phase
 Disappearance of retinal fluid ,
decrease in ant. Chamber and
vitreous cells , depigmentation
occurs in fundus giving ‘sunset
glow’ appearance.
 Depigmentation of corneal
limbus – Sigiura’s sign.
 In recurrent cases, anterior >
posterior uveitis.
 Choroidal neovascularisation in
peripapillary region and macula.
 RPE proliferation – subretinal
fibrosis.

8. Diagnosis & ancillary testing
 Early phase, FFA : numerous
hyperfluorescent dots at RPE.
 In chronic phase : diffusely
scattered dots of
hyperfluorescence d/t window
defects at RPE.
 Diffuse thickening of the
posterior choroid, serous
detachment of the retina,
vitreous opacities, posterior
thickening of the sclera

9.  Significant choroidal thickening along with subretinal
fluid .
OCT

10. Systemic associations
 Auditory : hearing loss,
tinnitus, vertigo.
 Neurological : meningitic
manifestations
 Dermal : vitiligo – hands,
face, shoulders & back.
 Poliosis & scalp alopecia.

11.  Uveitic Phase : granulomatous inflammation , tissue
inflammed by diffuse infiltration of lymphocytes,
macrophages, and epitheloid cells, and the exudates
of lymphocytes, macrophages .
 Immunohistochemical technique : choroidal infiltrate
composed of T lymphocytes.
 DF nodules
Pathology

12. 1. No history penetrating ocular trauma or surgery preceding initial onset
sympathetic uveitis.
2. No clinical or lab evidence s/o other ocular entities.
3. a) Early Manifestation – Evidence of diffuse Choroiditis with or without
Anterior uveitis, vitreous inflammatory reaction or Optic Disc Hyperaemia
which as focal area of subretinal fluid , bullous serous RD.
b) Bullous Serous RD
4. Neurological / Auditory : Meningismus, tinnitus, CSF pleocytosis
5. Integumentary : Alopecia, poliosis or vitiligo.
CompleteVKH : Criteria 1-5 must be present.
IncompleteVKH : Criteria 1-3 & either 4 or 5 must be present.
Revised Criteria for diagnosis of VKH

13.  Systemic corticosteroid : effective
 Prognosis is good , if diagnosed early.
 In recurrent cases, high doses of corticosteroids
should be given IV.
 Topical Ab and Cycloplegics for AC cells.
 Immunomodulators : If steroid therapy doesn’t work.
Treatment

14.  Although treated effectively by corticosteroid
therapy but may develop posterior subcapsular
cataract, secondary angle-closure glaucoma,
subretinal neovascularisation & subretinal fibrosis.
Course & outcome

15. Reference:
- Nussenblatt and whitecup
4th edition
- Myron yanoff 4th edition