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Sympathetic Ophthalmia

URGENT — Uveitis

Symptoms

  • bilateral pain and redness: onset in both the "injured" and "uninjured" eye
  • photophobia: extreme light sensitivity is often the earliest subjective symptom
  • decreased vision: typically bilateral drop in acuity (reading and distance)
  • loss of accommodation: difficulty focusing on near objects (early sign)
  • history: penetrating trauma or intraocular surgery (especially vitreoretinal) in one eye

Clinical Findings

Bilateral granulomatous panuveitis

  • mutton-fat kps: large, greasy-looking inflammatory cells on the corneal back surface
  • busacca/koeppe nodules: inflammatory lumps on the iris surface or pupillary margin
  • dalen-fuchs nodules: pathognomonic small, yellow-white nodules at the RPE level
  • vitritis: significant inflammatory "snow" in the vitreous cavity of both eyes

Posterior segment

  • sunset-glow fundus: chronic orange-red discoloration due to RPE depigmentation
  • serous retinal detachment: fluid pooling under the retina (similar to VKH)
  • choroidal thickening: diffuse inflammation of the vascular layer (best seen on EDI-OCT)
  • optic disc edema: swelling and hyperemia of the optic nerve head

Etiology

Pathophysiology

  • autoimmune response: T-cell mediated attack on previously "hidden" uveal (melanocyte) antigens
  • mechanism: trauma to one eye ("exciting eye") releases antigens into the bloodstream/lymphatics
  • result: the immune system attacks both the exciting eye and the healthy "sympathizing eye"

Timing

  • window: 90% occur within 1 year; most common between 2 weeks and 3 months post-injury
  • range: can occur as early as 5 days or as late as several decades after trauma

Red Flags

  • any uveitis in a healthy eye after fellow-eye trauma: must be SO until proven otherwise
  • bilateral serous detachments: a sign of intense choroidal inflammation requiring IV steroids
  • 14-day window: if a traumatized eye is blind and unsalvageable, enucleation within 14 days may prevent SO
  • relapse on taper: standard for SO; requires long-term (often lifelong) immunosuppression

Investigations

Imaging workup

  • fluorescein angiography (fa): shows pinpoint RPE leaks and late pooling (starry sky appearance)
  • icg angiography: hypocyanescent (dark) spots showing multifocal choroidal involvement
  • edi-oct: used to measure and track the characteristic thickening of the choroid

Systemic screening

  • chest x-ray / ct: to rule out sarcoidosis or tuberculosis (which mimic SO findings)
  • syphilis screen: RPR/FTA-ABS to rule out luetic panuveitis

Treatment

Induction

  • systemic steroids: high-dose oral Prednisone (1–2 mg/kg/day) or IV Methylprednisolone
  • topical steroids: Prednisolone Acetate 1% or Difluprednate q1-2h for anterior control

Maintenance (steroid-sparing)

  • immunomodulators: Methotrexate, Mycophenolate, or Azathioprine to allow steroid taper
  • biologics: Anti-TNF agents (e.g., Adalimumab) for refractory or severe cases

Prevention

  • enucleation (not evisceration): only if done within 14 days of trauma in a blind eye

Clinical Pearl

Sympathetic ophthalmia is the reason to consider enucleation of a blind painful eye within 2 weeks of trauma — once the sympathizing eye inflames, both eyes are at risk for life.

Differential Diagnosis

  • • vkh syndrome (identical ocular signs but NO history of trauma; has neuro/skin findings)
  • • sarcoidosis (bilateral granulomatous uveitis but with lung/systemic involvement)
  • • syphilis (the "great masquerader"; can present with panuveitis and RPE changes)
  • • phacoantigenic uveitis (reaction to lens protein; usually only in the injured eye)

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