Birdshot chorioretinopathy is a rare type of chronic inflammatory eye disease that can lead to vision loss or blindness. Common symptoms include floaters and blurred vision, usually in both eyes. Early diagnosis, treatment and monitoring are essential to protect your vision and prevent complications.
Birdshot chorioretinopathy (koor-ee-oh-reht-in-OHP-ah-thee), or BSCR, is a rare inflammatory eye disease. It’s a long-term (chronic) condition that can lead to permanent vision changes or complete loss of sight.
BSCR is a severe form of uveitis, a group of diseases that damage eye tissue. It causes swelling of the:
Hallmark signs of birdshot chorioretinopathy are cream-colored oval spots that are present in the deep retina and superficial (top layer) choroid. Early symptoms often involve floaters, blurred vision or both. The condition usually affects both eyes.
Anyone can develop birdshot chorioretinopathy, though people assigned female at birth may be slightly more likely to develop the condition. It most often affects middle-aged, white adults between the ages 40 and 60. It rarely affects children.
Several conditions, including other forms of uveitis, have symptoms that resemble birdshot chorioretinopathy. Getting an accurate diagnosis is crucial so you can get appropriate treatment. Conditions similar to BSCR include:
A group of inflammatory conditions known as white dot syndromes also share symptoms of BSCR. They cause damage (lesions) to the retina and choroid and include:
Birdshot chorioretinopathy is a relatively new disease, first described in 1949. In 1980, two eye specialists named it “birdshot chorioretinopathy” for its unique cream and orange oval-shaped spots scattered throughout the retina. The spots resemble the pattern from birdshot, small shotgun pellets (bullets).
Other names for birdshot chorioretinopathy are:
Birdshot chorioretinopathy is rare. Researchers believe it affects less than 1 in every 100,000 people.
More than 90% of people diagnosed with BSCR inherit (receive from their parents) a substance (antigen) called HLA-A29 that triggers an immune response. However, you don't need to have HLA-A29 to develop birdshot chorioretinopathy. You can also have the antigen and not get the disease.
Symptoms of birdshot chorioretinopathy can vary. It's a progressive disease with symptoms that typically worsen over several months to years.
Some people experience unusual changes in vision or perception. Symptoms may include:
In severe cases, symptoms may include cataracts or glaucoma, which can cause severe vision loss even after inflammation is treated. Birdshot chorioretinopathy may also lead to issues with the central retina, including cystoid macular edema and epiretinal membranes.
Diagnosis of birdshot chorioretinopathy can be difficult. It's a rare disease, and its unique spots may not be visible in early stages of the disease.
Healthcare providers may use a combination of tests to monitor you over time. Stay in touch with your provider to receive the care you need.
There's no cure for birdshot chorioretinopathy. But with timely diagnosis, treatment can reduce symptoms and prevent worsening of the disease.
Your care team recommends treatment, monitors you and adjusts medications based on your condition and any side effects. Your team may include:
Treatment for birdshot chorioretinopathy depends on the severity of your condition and how it changes over time. The goal of treatment is to reduce swelling in your eyes and prevent or reverse vision loss.
Healthcare providers typically first recommend high doses of corticosteroids to control inflammation in your eye. They then lower the dose as much as possible due to the risk of side effects. You may receive steroids through:
Many people also need long-term treatment with immunosuppressants. These drugs stop your immune system from attacking your eyes and may include one or more:
Long-term steroid and immunosuppressant use can cause serious side effects, including gastrointestinal or bone issues. They may also increase your risk of developing cataracts or glaucoma.
Your healthcare provider will monitor you for any side effects and prescribe other medications if needed. If you're taking steroids by mouth, providers will check you for bone damage (osteoporosis) and fracture risk.
Your provider may also recommend a bone density scan. You may receive medication to prevent bone loss (bisphosphonates).
Watch your condition for any changes or complications. Your healthcare provider will check your blood and urine to make sure your kidneys, liver and bone marrow are working as they should. You are also likely to have regular eye exams, including visual field tests and optical coherence tomography (OCT) scans.
There's no known way to prevent birdshot chorioretinopathy. In most cases, it's believed to be triggered by a specific antigen (a molecule that triggers an immune response) passed down in families.
Regular eye exams can help detect early symptoms, so you can receive timely treatment if needed. Tell your healthcare provider if any family members have eye disease.
The outlook for birdshot chorioretinopathy varies depending on the severity of the condition and the effectiveness of treatment. In milder cases, vision can remain stable or improve with treatment.
Birdshot chorioretinopathy requires prompt diagnosis and treatment from an ophthalmologist. Even if your vision is fine, an eye exam can detect low levels of inflammation.
Alert your healthcare provider to any side effects of medication or change in symptoms or vision. You may receive different medications as needed. Your healthcare provider can discuss options that work best for you.
A note from Cleveland Clinic
Birdshot chorioretinopathy is a rare yet treatable disease. Get regular eye checkups and follow your healthcare provider's recommendations. Over time, your provider will discuss ways you can continue to protect your vision and avoid complications, so you can see clearly for years to come.
Last reviewed by a Cleveland Clinic medical professional on 05/12/2022.
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