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Ophthalmology

Birdshot Chorioretinopathy: A Patient Guide

At a Glance

Birdshot chorioretinopathy is a rare, chronic inflammation of the retina and choroid that can affect side vision, color perception, and low-light vision before central acuity changes. Long-term imaging, functional testing, and immune-calming treatment help control inflammation and protect sight.

Birdshot Chorioretinopathy (BSCR) is a rare, chronic form of posterior uveitis, an inflammatory condition that affects the deep tissues at the back of the eye. It is a presumed autoimmune-mediated disease, meaning the body’s immune system mistakenly targets the choroid (the layer of blood vessels beneath the retina) and the retina itself. This condition is uniquely and strongly linked to a genetic marker called HLA-A29, which acts as a primary susceptibility factor. While the diagnosis may feel overwhelming, understanding that the disease requires long-term, specialized management rather than a quick fix is the first step toward protecting your vision [1][2].

Because Birdshot often leaves “sharp” central vision intact for many years, a standard eye chart exam is not enough to monitor the disease. The inflammation primarily impacts your functional vision—such as your side vision, color perception, and ability to see in low light—long before it affects your ability to read the 20/20 line. Protecting your sight requires multimodal imaging. This includes specialized dye tests like ICGA (to look deep into the choroidal layers) and FA (to evaluate retinal blood vessel circulation and leakage), alongside functional tests like ERGs and visual fields that measure how well your retina is performing electrically [3][4]. No single test captures everything, and not every test is needed at every visit; your doctor will tailor the testing schedule to you.

The journey from diagnosis to stability involves a structured treatment strategy designed to “quiet” the immune system. While powerful corticosteroids are often used initially to stop active inflammation, the long-term goal is typically to transition to steroid-sparing immunomodulatory therapies (and in some cases, biologics). These medications aim to achieve sustained remission—suppressing the disease to reduce the risk of further tissue damage. This process requires a dedicated care team, usually led by a uveitis specialist, who can adjust your treatment based on the subtle shifts in your diagnostic scans [5][6].

Living with Birdshot means establishing a partnership with your medical team based on consistent monitoring. What to do now: Understand that active Birdshot requires prompt treatment, never stop your medications without your doctor’s guidance, and always contact your care team immediately if you experience sudden visual changes [7][8].

Common questions in this guide

What is Birdshot chorioretinopathy, and what does HLA-A29 have to do with it?
Birdshot chorioretinopathy is a rare, long-lasting inflammation affecting the retina and choroid at the back of the eye. HLA-A29 is a genetic marker strongly associated with the condition, but specialists also use eye examinations, imaging, and functional tests when evaluating the disease.
Can my eye chart look normal even if Birdshot is active?
Yes. Birdshot can leave sharp central vision, including the 20/20 line, intact while affecting side vision, color perception, or the ability to see in low light. That is why eye doctors use imaging and functional testing in addition to a standard vision chart.
Which tests are used to monitor Birdshot chorioretinopathy?
Monitoring may include ICGA and FA, which use specialized imaging to assess the choroid and retinal blood vessels, as well as ERGs and visual-field testing to measure retinal function. No single test shows every aspect of the disease, so your doctor will tailor the testing schedule to your needs.
How is Birdshot chorioretinopathy treated?
Corticosteroids are often used first to control active inflammation. The longer-term plan commonly involves changing to steroid-sparing medicines that regulate the immune system, with biologic medicines used in some cases to help maintain remission.
How will my doctor know whether Birdshot is in remission?
Your care team will look for signs that inflammation is quiet on multimodal imaging and that functional tests are stable or improving. Because Birdshot can require long-term control, remission is followed with ongoing specialist monitoring rather than a single test or visit.
What should I do if I notice a sudden change in my vision?
Contact your eye-care team immediately, because sudden visual changes may need prompt assessment. Do not stop or change your Birdshot medicines on your own; follow the medication plan provided by your doctor.

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Questions to Ask Your Doctor

Curated prompts to bring to your next appointment.

  1. 1.How much experience do you have managing Birdshot specifically, and do you frequently coordinate care with a rheumatologist?
  2. 2.Which functional test—the ERG or the visual field—do you consider the most sensitive indicator of my disease activity?
  3. 3.What are the specific signs of 'remission' we are looking for in my multimodal imaging over the coming year?
  4. 4.Can you explain the long-term plan for transitioning me from steroids to a steroid-sparing maintenance medication?

References

References (8)
  1. 1

    Birdshot chorioretinopathy: current knowledge and new concepts in pathophysiology, diagnosis, monitoring and treatment.

    Minos E, Barry RJ, Southworth S, et al.

    Orphanet journal of rare diseases 2016; (11(1)):61 doi:10.1186/s13023-016-0429-8.

    PMID: 27175923
  2. 2

    Guidelines From the French-Speaking Society for Histocompatibility and Immunogenetics (SFHI) for Harmonisation of HLA Genotyping in Autoimmune Diseases, Drug Hypersensitivity and Pharmacogenetics.

    Renac V, Moalic V, Dautin G, et al.

    HLA 2025; (106(4)):e70442 doi:10.1111/tan.70442.

    PMID: 41137743
  3. 3

    Evidence and Consensus-based Imaging Guidelines in Birdshot Chorioretinopathy: Multimodal Imaging in Uveitis (MUV) Taskforce Report 8.

    Pichi F, Miserocchi E, Grewal DS, et al.

    American journal of ophthalmology 2025; (278()):271-281 doi:10.1016/j.ajo.2025.06.029.

    PMID: 40545009
  4. 4

    Five-Year Trends in Multifocal Electroretinogram for Patients With Birdshot Chorioretinopathy.

    Mailhac A, Labarere J, Aptel F, et al.

    American journal of ophthalmology 2019; (200()):138-149 doi:10.1016/j.ajo.2018.11.022.

    PMID: 30557530
  5. 5

    Prognostic Factors of Treatment Failure with First-Line Corticosteroid-Sparing Agents in Patients with Birdshot Chorioretinopathy: A Retrospective Study.

    Goupillou P, Leclercq M, Muraine M, et al.

    Ocular immunology and inflammation 2025; (33(7)):1298-1302 doi:10.1080/09273948.2025.2491570.

    PMID: 40262577
  6. 6

    Treatment Outcomes in Birdshot Chorioretinitis: Corticosteroid Sparing, Corticosteroid Discontinuation, Remission, and Relapse.

    Crowell EL, France R, Majmudar P, et al.

    Ophthalmology. Retina 2022; (6(7)):620-627 doi:10.1016/j.oret.2022.03.003.

    PMID: 35283323
  7. 7

    Long-term outcomes of systemic corticosteroid-sparing immunomodulatory therapy for Birdshot Retinochoroidopathy.

    You C, Lasave AF, Kubaisi B, et al.

    Ocular immunology and inflammation 2020; (28(6)):966-974 doi:10.1080/09273948.2019.1641610.

    PMID: 31567006
  8. 8

    Outcomes of "Early" Withdrawal of Corticosteroid Sparing Immunomodulatory Therapy for Birdshot Retinochoroidopathy.

    You C, Meese H, Stephenson A, et al.

    Ocular immunology and inflammation 2019; (27(7)):1165-1173 doi:10.1080/09273948.2018.1506040.

    PMID: 30207810

This page explains Birdshot chorioretinopathy, testing, treatment, and monitoring for informational purposes only and does not constitute medical advice. Your uveitis specialist should interpret your results and guide medication decisions.