Sympathetic Ophthalmia
๐๏ธโ๐จ๏ธ Sympathetic ophthalmia is a rare autoimmune condition. It famously occurred in Louis Braille (inventor of braille) after an eye injury, leading to bilateral blindness.
๐ About
- An autoimmune, type IV hypersensitivity reaction.
- Triggered by penetrating trauma or intraocular surgery to one eye.
- Results in autoimmune inflammation of the fellow (sympathising) eye.
๐งฌ Aetiology
- A granulomatous inflammatory response to melanin-containing ocular structures (e.g. photoreceptor outer segments).
- Develops after exposure of uveal antigens to the immune system following trauma or surgery.
๐ฉบ Clinical Features
- History of penetrating trauma or intraocular surgery.
- Typically presents as a bilateral granulomatous panuveitis.
- Onset usually ~3 months after injury but can be delayed for years.
- Symptoms: pain, photophobia, conjunctival injection, marked โ visual acuity.
- Signs: mutton-fat keratic precipitates, Dalen-Fuchs nodules (epithelioid cell clusters between RPE and Bruchโs membrane).
๐ Investigations
- Diagnosis is largely clinical (history + findings).
- OCT: assesses complications (retinal detachment, macular oedema).
- Fluorescein angiography: may show areas of inflammation or leakage.
๐ Management
- High-dose IV methylprednisolone in acute phase.
- Long-term immunosuppression (azathioprine, ciclosporin, cyclophosphamide).
- Enucleation of a painful, non-seeing injured eye may prevent further autoimmune activation.
๐ Prognosis
- Vision loss may be severe โ complete bilateral blindness is possible.
- With prompt treatment, systemic immunosuppression can preserve some visual function in many patients.
๐ References