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Vogt–Koyanagi–Harada Syndrome

URGENT — Uveitis

Symptoms

  • sudden vision loss: typically bilateral and profound
  • prodrome: flu-like symptoms, severe headache, and a stiff neck (meningismus)
  • auditory: tinnitus (ringing in ears), muffled hearing, or vertigo
  • sensitivity: photophobia and eye pain
  • skin/hair: patches of white hair (poliosis) or skin (vitiligo) appearing later

Clinical Findings

Phase 1: prodromal (the "brain" phase)

  • mimicry: looks like viral meningitis; patient has fever, headache, and ear pain

Phase 2: acute uveitic (the "eye" phase)

  • serous retinal detachments: pathognomonic multiple "pockets" of fluid lifting the retina
  • panuveitis: inflammation in the front (cells/flare) and back (vitritis) of the eye
  • thickened choroid: the vascular layer under the retina becomes massively swollen

Phase 3: convalescent (the "skin" phase)

  • sunset-glow fundus: the retina turns a striking orange-red due to loss of pigment
  • sugiura sign: whitening of the perilimbal conjunctiva
  • poliosis/vitiligo: whitening of eyelashes, eyebrows, or skin patches

Phase 4: chronic recurrent

  • dalen-fuchs nodules: small yellow-white spots in the peripheral retina

Etiology

Pathophysiology

  • autoimmune attack: T-cells mistakenly attack melanocytes (pigment-producing cells)
  • targets: anywhere pigment is found—the uvea (eye), inner ear, meninges (brain cover), and skin

Risk factors

  • ancestry: most common in Asian, Hispanic, Middle Eastern, and Native American populations
  • genetics: strongly associated with HLA-DR4 and HLA-DRB1*04:05

Red Flags

  • headache + vision loss: bilateral serous detachments with a headache is VKH until proven otherwise; requires immediate IV steroids
  • stiff neck: indicates meningeal involvement; requires neuro-consult and potentially an LP
  • rapid recurrence: stopping steroids too early (before 6 months) almost guarantees a relapse
  • sunset-glow: indicates the disease has entered a chronic stage and needs long-term immunosuppression

Investigations

Imaging

  • oct macula: shows "septated" pockets of subretinal fluid (looks like bridges over water)
  • fluorescein angiography (fa): "starry sky" appearance—pinpoint leaking dots across the retina
  • icg angiography: shows multiple dark spots (hypocyanescent) representing choroidal inflammation

Systemic workup

  • lumbar puncture: CSF pleocytosis (elevated white cells) supports the diagnosis
  • rule-outs: must exclude Syphilis, TB, and Sarcoid before starting heavy steroids

Treatment

Acute phase

  • iv methylprednisolone: 1g daily for 3 days to save vision
  • oral prednisone: 1–2 mg/kg/day with an extremely slow taper (over 6+ months)

Long-term control

  • steroid-sparing therapy: Mycophenolate Mofetil or Methotrexate to prevent the "sunset-glow" stage
  • biologics: Anti-TNF agents (Adalimumab) for refractory cases

Clinical Pearl

Bilateral multifocal serous retinal detachments in a pigmented patient with headache and tinnitus is VKH — ICG shows dark dots, and aggressive long-term steroids prevent the sunset-glow fundus.

Differential Diagnosis

  • • sympathetic ophthalmia (identical presentation, but requires a history of eye trauma/surgery)
  • • posterior scleritis (usually unilateral and very painful; shows a "T-sign" on ultrasound)
  • • central serous chorioretinopathy (CSCR) (fluid pockets but NO inflammation or headache)

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