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Sympathetic ophthalmia

After a penetrating injury or surgery to one eye, the immune system attacks both eyes. A rare but blinding bilateral uveitis.

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What it is

A rare bilateral granulomatous panuveitis that follows a penetrating injury or surgery to one eye. Exposure of hidden uveal antigens triggers an autoimmune response that inflames both the injured (exciting) eye and the fellow (sympathising) eye, weeks to years later. It threatens sight in the only healthy eye.

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Who gets it

Rare; most cases begin within the first year after the injury or surgery, many within three months. Vitreoretinal surgery is now a common trigger.

Causes

  • Penetrating eye trauma
  • Intraocular surgery, especially repeated vitreoretinal surgery

Risk factors

  • Penetrating injury with uveal prolapse
  • Multiple eye operations

Pathophysiology

Uveal and retinal antigens normally hidden from the immune system are exposed by the wound; a T-cell-mediated response then targets the uvea of both eyes, forming granulomas in the choroid.

Symptoms and signs

  • Blurred vision and light sensitivity in the uninjured eye
  • Pain and redness in both eyes
  • Floaters
  • Sometimes hearing changes, vitiligo or poliosis

Diagnosis

Clinical
Bilateral granulomatous panuveitis after a history of penetrating injury or surgery to one eye; small yellow-white choroidal spots may be seen.
Tests
Exclusion of infection (syphilis, tuberculosis) and other uveitides.
Imaging
OCT and fluorescein angiography show choroidal involvement and serous detachments; ultrasound shows choroidal thickening.

Differential diagnosis

  • Vogt-Koyanagi-Harada disease (no trauma)
  • Post-operative endophthalmitis
  • Lens-induced uveitis
  • Sarcoidosis

Goal of treatment

Suppress the inflammation in both eyes quickly and for long enough to save vision.

Treatment

Medical
High-dose systemic corticosteroids tapered slowly, with long-term immunosuppressive or biologic drugs.
Surgical
Removal of a blind, severely injured eye within about two weeks of injury is considered to reduce the risk; once disease has started it does not reliably help.
Follow-up
Lifelong follow-up of the fellow eye after penetrating eye injury.

Complications

  • Cataract
  • Glaucoma
  • Retinal detachment
  • Bilateral blindness

Prognosis

Most patients keep useful vision with early and sustained immunosuppression.

Prevention

  • Prompt, careful repair of penetrating injuries
  • Early discussion of removing a blind, disorganised injured eye

Test yourself

Think of the answer, then tap the question to see it.

What precedes sympathetic ophthalmia?

A penetrating injury or intraocular surgery to one eye.

Which eye is at risk?

Both eyes, including the uninjured fellow eye.

How is it treated?

With high-dose corticosteroids and long-term immunosuppression.

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Educational information, not medical advice. For any symptom, see a health professional.

Sources: Sympathetic Ophthalmia (StatPearls Publishing (NCBI Bookshelf), 2024).

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