Sympathetic Ophthalmia

How sympathetic ophthalmia develops

The inside of the eye is normally shielded from the immune system. A penetrating injury or surgery can expose proteins from the uvea and retina (such as melanin-related antigens) to the immune system through the lymphatic system of the conjunctiva. The immune system then recognizes these proteins as foreign and launches a T-cell attack on the uvea of both eyes.

Triggers include:

• penetrating eye injuries, especially those involving the uvea or with retained tissue
• eye surgery: vitrectomy and other retinal surgery, cataract surgery, glaucoma surgery and laser procedures, particularly after multiple operations
• ruptured corneal ulcers, rarely

Genetic factors, including certain HLA types, may make some people more susceptible.

Symptoms

In the uninjured (sympathizing) eye:

• blurred vision, especially difficulty with near vision early on
• sensitivity to light
• eye pain and redness
• floaters

In the injured (exciting) eye: worsening inflammation and vision.

Some people also have features similar to Vogt-Koyanagi-Harada disease: headache, ringing in the ears, hearing loss, patches of white hair or eyelashes, and pale patches of skin.

Diagnosis

Sympathetic ophthalmia is a clinical diagnosis: bilateral uveitis after injury or surgery to one eye. An ophthalmologist uses:

• slit-lamp and dilated retinal examination: inflammation in the front and back of both eyes, with small yellow-white spots in the deep retina (Dalen-Fuchs nodules)
• optical coherence tomography (OCT): retinal swelling and fluid
• fluorescein and indocyanine green angiography: inflammation of the choroid
• ultrasound when the view is blocked
• tests to exclude infection, such as tuberculosis and syphilis, and other causes of uveitis

Treatment

• Corticosteroids: high-dose oral or IV steroids at first, with steroid eye drops and drops to dilate the pupil. The dose is tapered slowly over months.
• Steroid-sparing immunosuppression: methotrexate, mycophenolate, azathioprine, cyclosporine or tacrolimus to maintain control and limit steroid side effects.
• Biologic therapy: adalimumab or infliximab for resistant disease.
• Local injections or implants of steroids into the eye in some cases.
• Long-term monitoring for complications.

Removing the injured eye

Removing (enucleating) a severely injured eye with no useful vision within about 2 weeks of the injury may reduce the risk of sympathetic ophthalmia. This is considered only when the injured eye has no visual potential. Once the disease has started, removing the injured eye has not been shown to help reliably, and the injured eye may end up with better vision than the other.

Complications

• cataract
• glaucoma
• macular swelling and scarring
• retinal detachment
• abnormal blood vessel growth under the retina
• shrinking of the eye in severe cases
• side effects of long-term steroids

Outlook

Before steroids, sympathetic ophthalmia often caused blindness in both eyes. With early, aggressive treatment, most people now keep useful vision, and many achieve good vision, though long-term treatment and monitoring are usually needed, and relapses can occur.

Prevention

• wear protective eyewear for risky work, sports and hobbies
• get urgent repair of penetrating eye injuries
• regular follow-up after eye injury or multiple eye surgeries
• report any symptoms in the uninjured eye promptly

Frequently asked questions

What is sympathetic ophthalmia?

Sympathetic ophthalmia is a rare inflammation of both eyes that develops after a penetrating injury or surgery to one eye. The immune system reacts to eye proteins exposed by the injury and attacks the uveal tissue in both eyes, threatening vision.

How soon after an eye injury does sympathetic ophthalmia occur?

It most often appears within 3 months of the injury, and the vast majority of cases occur within a year. However, it has been reported weeks to decades later, so anyone with a previous eye injury should report new symptoms in either eye.

What are the symptoms of sympathetic ophthalmia?

Symptoms include blurred vision, difficulty focusing up close, light sensitivity, eye pain, redness and floaters in the uninjured eye, along with worsening inflammation in the injured eye. Some people also have headaches, hearing changes or white patches of hair or skin.

How is sympathetic ophthalmia treated?

Treatment starts with high-dose corticosteroids, given by mouth, IV, drops or injections, followed by long-term immune-suppressing drugs or biologics such as adalimumab. Close follow-up with an ophthalmologist is essential.

Can sympathetic ophthalmia be prevented?

The best prevention is avoiding eye injuries with protective eyewear and getting prompt surgical repair of penetrating injuries. In rare cases where an injured eye has no chance of useful vision, early removal of that eye may reduce the risk.