Vogt-Koyanagi-Harada disease is an autoimmune reaction to melanocytes that causes bilateral uveitis and can involve other organs. It has three phases: a prodromal phase with influenza-like symptoms, an uveitic phase with ocular inflammation and retinal detachment, and a chronic phase with depigmentation and recurrence of inflammation. Diagnosis involves evaluating for ocular inflammation as well as neurological, auditory, and integumentary symptoms. Treatment is with systemic corticosteroids, though recurrence and complications are possible.

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