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Describe Vogt-Koyanagi-Harada disease and the other specific uveitic syndromes.

Ophthalmology · 3rd Professional

Vogt–koyanagi–harada Disease

  • VOGT-koyanagi-HARADA (VKH) disease is a bilateral, diffuse, granulomatous panuveitis with exudative retinal detachment, accompanied by neurological, auditory and cutaneous features.
  • It is an autoimmune reaction against melanocytes — which explains why every tissue affected is one that contains them.
  • Epidemiology — affects pigmented races: Asians (including Indians), Hispanics, Middle Eastern and Native American peoples; and is rare in Europeans. Commoner in women, in the third to fifth decade. Associated with HLA-DR4 and HLA-DRB1*0405
StageFeatures
1. Prodromal (a few days)
  • Resembles a viral illness: headache, fever, nausea, meningism and neck stiffness; tinnitus, vertigo and hearing loss; and cerebrospinal fluid pleocytosis.
  • The ocular disease has not yet begun, and the patient may be seen by a physician or a neurologist
2. Acute uveitic (weeks)
  • Sudden bilateral blurring; a diffuse choroiditis with multifocal serous detachments coalescing into a bullous exudative retinal detachment; disc hyperaemia and oedema; and a granulomatous anterior uveitis with mutton-fat KPs.
  • Fluorescein angiography shows multiple pinpoint leaks at the level of the pigment epithelium with pooling in the subretinal space — a characteristic pattern
3. Convalescent (months)The detachment resolves and depigmentation appears: the classical sunset glow fundus — a diffuse orange-red depigmented fundus — with sugiura’S sign (perilimbal vitiligo, seen early and particularly in Japanese patients), vitiligo, poliosis (whitening of the lashes, brows and hair) and alopecia
4. Chronic recurrentRecurrent anterior uveitis with complications — cataract, glaucoma, choroidal neovascularisation and subretinal fibrosis

Treatment of VKH and Related Disorders

  • Treatment of VKH — early, high-dose and prolonged systemic corticosteroid: intravenous methylprednisolone followed by oral prednisolone 1 to 2 mg/kg, tapered very slowly over at least 6 months. Early and adequate treatment prevents the chronic recurrent stage, and under-treatment or rapid tapering is the commonest cause of a poor outcome. Steroid-sparing immunosuppression — ciclosporin, azathioprine, mycophenolate or a biological agent — is added early in most modern protocols
  • The prognosis is good with prompt aggressive treatment, and this is worth emphasising, since the acute presentation with bilateral detachments is alarming
Related disorderFeatures
BEHCET disease
  • An occlusive vasculitis affecting the Silk Road populations — Turkey, Iran, Central Asia, Japan — associated with HLA-B51.
  • The ocular hallmark is a recurrent, explosive, non-granulomatous panuveitis with a mobile, shifting hypopyon in a relatively white eye, and an occlusive retinal vasculitis affecting both arteries and veins.
  • Systemic: recurrent oral and genital ulceration, erythema nodosum, pseudofolliculitis and a positive pathergy test.
  • Blinding if untreated; requires early aggressive immunosuppression — azathioprine, ciclosporin, interferon alfa or infliximab — since steroid alone is inadequate
FUCHS heterochromic iridocyclitis
  • A chronic, low-grade, unilateral iridocyclitis with a characteristically quiet, white eye and NO pain or redness.
  • Diffuse stellate KPs over the whole endothelium; diffuse iris atrophy causing heterochromia (the affected iris the lighter); and, importantly, NO posterior synechiae.
  • Complications: cataract (very common) and glaucoma.
  • A filiform haemorrhage from the angle on paracentesis is AMSLER’S sign.
  • Associated with rubella.
  • Steroids are largely ineffective and should be avoided; cataract surgery does well
POSNER-schlossman syndrome
  • Glaucomatocyclitic crisis — recurrent unilateral attacks of markedly raised pressure (40 to 60 mmHg) with minimal inflammation, a few fine KPs, a mid-dilated pupil and an open angle.
  • Self-limiting, but recurrent attacks cause glaucomatous damage
Juvenile idiopathic arthritis
  • Chronic, bilateral, non-granulomatous anterior uveitis in a white, painless, asymptomatic eye — so it is detected only by screening.
  • Highest risk in young girls with pauciarticular disease who are ANA-positive.
  • Complications: band keratopathy, cataract and glaucoma.
  • Regular slit-lamp screening on a defined schedule is mandatory

Applied Aspects

  • Treat VKH early, hard and long; the prognosis is good with prompt high-dose steroid and poor with hesitant treatment or a rapid taper
  • Ask about headache, tinnitus and hearing in bilateral panuveitis with serous detachments; the prodrome may already have been dismissed as a viral illness
  • A shifting hypopyon in a white eye means Behcet; ask about oral and genital ulcers, and refer for immunosuppression rather than relying on steroid

All questions in Diseases of the Uveal Tract

  1. Q1Describe the anatomy of the uveal tract. Classify uveitis and describe anterior uveitis with its management. ★
  2. Q2Describe intermediate and posterior uveitis with their causes and investigation. ★
  3. Q3Describe the complications of uveitis and the principles of its management. ★
  4. Q4Describe the tumours of the uveal tract. ★
  5. Q5Describe endophthalmitis and panophthalmitis with their management. ★
  6. SN1Describe sympathetic ophthalmitis with its prevention and treatment. ★
  7. SN2Describe keratic precipitates and the other signs of uveitis. ★
  8. SN3Describe ocular tuberculosis and sarcoidosis. ★
  9. SN4Describe ocular toxoplasmosis with its features and management. ★
  10. SN5Describe Vogt-Koyanagi-Harada disease and the other specific uveitic syndromes. ★
  11. SN6Describe uveal coloboma and the congenital anomalies of the iris. ★
  12. SN7Describe HLA-B27 associated uveitis and uveitis in the immunosuppressed.

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