Vitiliginous Chorioretinitis

Vitiliginous chorioretinitis is a long-lasting inflammation in the back of the eye. It mainly affects the choroid (the blood-rich layer under the retina) and the retina itself. Doctors see many pale, oval, cream-colored spots in both eyes. These spots are scattered in a pattern that looks like “birdshot,” which is why the modern name is birdshot chorioretinopathy (BCR) or HLA-A29 uveitis. The disease is rare, often starts in middle age, and can slowly get worse if untreated. BioMed Central+2EyeWiki+2 “Vitiliginous” refers to the pale color of the lesions (like vitiligo on the skin). The pale color comes from damage and pigment loss in the choroid and retina. Orpha

Almost all patients carry a specific tissue type called HLA-A29. This strong link suggests an autoimmune disease where the immune system—likely certain T cells (including Th17 cells)—attacks structures in the back of the eye. Genes that shape antigen presentation (ERAP1/ERAP2) also seem to raise risk in HLA-A29-positive people. PMC+2PMC+2

Vitiliginous chorioretinitis is a rare, long-lasting inflammation inside both eyes that mainly affects the retina and the choroid (the blood-rich layer under the retina). Doctors nicknamed it “birdshot” because the pale, cream-colored spots look like scatter from a shotgun on the back of the eye. Most patients carry a tissue marker called HLA-A29, and the disease behaves like an autoimmune condition where the immune system mistakenly attacks eye tissue. Without proper treatment, it can slowly reduce night vision, cause floaters, blur central vision, and narrow side vision. Diagnosis uses eye exam plus imaging tests like fluorescein and indocyanine green angiography, optical coherence tomography (OCT), fundus autofluorescence, and electroretinography (ERG). Treatment aims to calm inflammation, protect the macula and optic nerve, and preserve vision using corticosteroids and steroid-sparing immunomodulatory therapy; targeted biologics and intraocular steroid implants are often needed. PMC+4PMC+4MDPI+4

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Other names

• Birdshot chorioretinopathy / birdshot retinochoroidopathy / birdshot chorioretinitis

• HLA-A29 uveitis

• Vitiliginous choroiditis (older term)
  All these terms describe the same clinicopathologic entity. Orpha

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Types

There is no rigid “official” subtype list, but clinicians often describe patterns that help in care and research:

1. Classic lesion-predominant type
   People have the hallmark cream-colored, oval choroidal spots in both eyes. Vitritis (cells in the vitreous) is mild to moderate. This is the pattern many textbooks show. EyeWiki

2. Vasculitis-predominant type
   Retinal blood vessels are more inflamed, with leakage on fluorescein angiography and variable optic nerve involvement. Patients may notice blurred or fluctuating vision from macular edema even when the spots are subtle. Taylor & Francis Online

3. ICGA-dominant (imaging-positive) early type
   Indocyanine-green angiography (ICGA) shows many hypofluorescent spots even when ophthalmoscopy shows few or no visible lesions. This pattern can reveal disease earlier. PMC+1

4. HLA-A29-positive “typical” BCR vs. “birdshot-like” (HLA-A29-negative)
   Most patients are HLA-A29 positive. Rare “birdshot-like” cases lack HLA-A29, and experts debate whether these are the same disease or a mimic. Current classification does not require HLA-A29 for diagnosis, but its presence strongly supports it. PMC+1

5. Practical clinical pattern

   1. Early inflammatory phase – active vitreous cells, vascular leakage on fluorescein angiography (FA), many hypofluorescent lesions on ICGA, and ERG delays may appear before extensive visible spots. AAO Journal+2JAMA Network+2

   2. Choroid-dominant pattern – numerous ICGA dark spots far exceeding what is seen clinically; this helps detect disease activity and monitor treatment response. PubMed+1

   3. Retina-dominant pattern – cystoid macular edema (CME), epiretinal membrane, retinal vasculitis, and ellipsoid zone changes on OCT; these drive vision loss. University of Utah Healthcare

   4. Quiescent/atrophic phase – lesions become atrophic with RPE/outer retinal loss, ERG amplitudes fall, and visual fields constrict; inflammation can smolder for years. PubMed+1

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Causes

The true root cause is not fully known. The following items explain proven risks, immune pathways, and plausible triggers that researchers discuss. Each entry is short and plain.

1. HLA-A29 genetic risk – The major risk. Most patients carry this allele; it is a hallmark of the disease. PMC

2. ERAP2 variants – Changes in ERAP2 can alter peptide trimming and presentation to HLA-A29, shaping autoimmune responses. Wikipedia

3. ERAP1 variants – Similar to ERAP2, ERAP1 polymorphisms may modify antigen processing and risk. Wikipedia

4. Autoreactive T-cells – Evidence points to T-cell–driven inflammation against ocular antigens. PMC

5. Th17 pathway / IL-17 – Elevated or reactive Th17 cells and IL-17 have been reported in patients, supporting an inflammatory axis. PMC+1

6. Antigen presentation dysregulation – The HLA-A29–ERAP axis suggests abnormal presentation of self-peptides to T cells. Wikipedia

7. Age (middle decades) – Typical onset is in the 40s–60s. Age likely reflects immune and environmental exposures over time. BioMed Central

8. Ancestry – Disease is reported more often in individuals of Northern/Western European ancestry, reflecting HLA-A29 distributions. BioMed Central

9. Autoimmune bias – The strong HLA class I link and chronic course fit an autoimmune mechanism. PMC

10. Retinal/choroidal immune privilege breakdown – Local immune changes may expose retinal antigens. (Inference from autoimmune uveitis models aligned with BCR literature.) PMC

11. Cytokine network (IL-6, TNF, others) – Pro-inflammatory cytokines participate in posterior uveitis, including BCR. BioMed Central

12. Retinal vasculitis component – Vessel inflammation is part of the disease process and can fuel edema and damage. ophthalmologyscience.org

13. Genetic background beyond ERAP – Polygenic factors likely modulate risk, though HLA-A29 dominates. PMC

14. Environmental triggers (hypothesized) – Triggers such as infections have been considered but not proven; clinicians mainly rule out mimics. BioMed Central

15. Delayed diagnosis – Not a cause of onset, but late diagnosis allows inflammation to persist and worsen vision. BioMed Central

16. Impaired choroidal circulation – Choroidal hypoperfusion is seen on imaging and may perpetuate tissue injury. PubMed

17. Photoreceptor stress – Cone system dysfunction on ERG suggests photoreceptor involvement over time. PMC

18. Macular edema – Inflammatory leakage at the macula sustains blurred vision and can amplify damage. Review of Ophthalmology

19. Optic nerve inflammation – Optic disc edema or leakage can occur and contribute to visual symptoms. Taylor & Francis Online

20. Immune memory – Once established, autoreactive memory responses may drive the chronic, relapsing course. (Inference consistent with autoimmune uveitis behavior.) PMC

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Symptoms

1. Blurry vision – Vision is not sharp, especially for fine detail or reading. Macular swelling is a common reason. Review of Ophthalmology

2. Floaters – Small gray or black specks drift in the vision due to cells and debris in the vitreous. Review of Ophthalmology

3. Photopsias (flashes) – Brief flickers or flashes of light from retinal irritation. Review of Ophthalmology

4. Night vision trouble (nyctalopia) – Seeing in dim light is hard because the diseased retina and choroid cannot support normal function. Review of Ophthalmology

5. Colors look faded – Color sensitivity drops as cone cells are affected. PMC

6. Paracentral blind spots – Small dark areas near the center of vision make reading lines or faces difficult. Review of Ophthalmology

7. Light sensitivity (photophobia) – Bright light is uncomfortable due to inflamed tissue. BioMed Central

8. Distortion (metamorphopsia) – Straight lines look wavy because the macula is swollen or disrupted. Review of Ophthalmology

9. Difficulty with contrast – Gray-on-gray or low-contrast objects are hard to see. BioMed Central

10. Glare problems – Oncoming headlights or sunlight cause major visual discomfort. BioMed Central

11. Reduced visual field – Side or near-central field may shrink as lesions expand. BioMed Central

12. Dark adaptation delay – Eyes take longer to adjust after lights go off. BioMed Central

13. Headache or eye ache – Some patients feel pressure or ache from inflammation. (General uveitis symptom consistent with BCR.) BioMed Central

14. Reading fatigue – Sustained reading becomes tiring because of blur, distortion, and poor contrast. BioMed Central

15. Fluctuating vision – Symptoms can wax and wane as inflammation changes. BioMed Central

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Diagnostic tests

A) Physical examination (at the slit lamp and dilated fundus)

1. Visual acuity – Read eye charts to measure clarity. A drop in lines suggests macular involvement or active disease. Review of Ophthalmology

2. Pupil and color checks – Test color plates and pupillary reflexes; reduced color vision hints at cone dysfunction. PMC

3. Intraocular pressure (IOP) – Check pressure; treatment and uveitis can affect IOP. (Standard uveitis care principle.) BioMed Central

4. Anterior segment exam – Usually shows absent to mild cells; heavy keratic precipitates or synechiae argue against classic birdshot. EyeWiki

5. Dilated fundus exam – The key step: look for the many, oval, cream lesions in both eyes and signs of vasculitis or disc leakage. EyeWiki

B) Manual/functional chairside tests

6. Amsler grid – A hand-held grid detects central wavy lines or missing spots. Useful for daily self-monitoring too. (Common macular test aligned with BCR monitoring.) BioMed Central

7. Contrast sensitivity charts – Identify low-contrast letters; reduced scores match patient complaints even when acuity seems okay. BioMed Central

8. Color vision plates (Ishihara or D-15) – Simple color tests document cone pathway problems. PMC

9. Dark adaptation assessment – Chairside tools or history confirm slow adaptation; reflects photoreceptor/choroidal issues. BioMed Central

10. Confrontation visual fields – A quick check for field defects to guide formal perimetry. BioMed Central

C) Laboratory and pathological tests (to support diagnosis and exclude mimics)

11. HLA-A29 typing – A positive result strongly supports BCR but is not mandatory in new classification criteria. PMC

12. Syphilis serology (VDRL/RPR and treponemal tests) – Rules out infectious uveitis that can mimic lesions. (Standard posterior uveitis exclusion.) BioMed Central

13. Tuberculosis testing (IGRA/PPD + chest imaging if indicated) – Excludes TB-related uveitis. (Standard exclusion in uveitis workups.) BioMed Central

14. Sarcoidosis screening (ACE, chest imaging if indicated) – Another important mimic to exclude when lesions are atypical. (General uveitis guidance consistent with BCR reviews.) BioMed Central

15. Autoimmune panels as guided (ANA, etc.) – Used to look for systemic autoimmune disease when the picture is unclear. (General uveitis practice aligned with reviews.) BioMed Central

D) Electrodiagnostic tests (objective function)

16. Full-field ERG (electroretinogram) – Often shows cone system dysfunction and reduced responses. Helpful for baseline and follow-up. PMC

17. Multifocal ERG – Maps central retinal function and can track macular recovery or decline. (Used in posterior uveitis including BCR.) PMC

18. Pattern ERG/EOG (as available) – Additional measures of macular and retinal pigment epithelium function to complement ERG. (Supplemental electrodiagnostics referenced in reviews.) PMC

E) Imaging tests

19. Optical coherence tomography (OCT) – Cross-section scans show macular edema, outer retinal damage, and retinal thinning over time. Guides treatment. Taylor & Francis Online

20. Fluorescein angiography (FA) and Indocyanine-green angiography (ICGA) – FA shows vessel leakage and disc staining; ICGA highlights many hypofluorescent choroidal spots and can be more sensitive than the exam in early disease. Together, they define activity and help confirm diagnosis. PMC+1

Non-pharmacological treatments (therapies & others)

1. Regular specialist follow-up with a uveitis team
   Purpose: Catch inflammation early, measure treatment effect, and prevent irreversible damage.
   Mechanism: Scheduled exams, visual fields, OCT, FA/ICGA, and ERG quantify inflammation and function. Tighter control means less macular edema and less choroidal damage over time. Evidence-based care uses multimodal imaging plus ERG to guide when to raise or reduce therapy. Ajo+1

2. ICGA-guided monitoring
   Purpose: Reveal choroidal lesions “hidden” on routine exam so therapy can be timed better.
   Mechanism: Indocyanine green binds plasma proteins and images choroidal circulation. In birdshot, many hypofluorescent spots (active lesions) appear and resolve with treatment; ICGA is sensitive for activity and relapse surveillance. PubMed+1

3. OCT-based macular care
   Purpose: Detect and track cystoid macular edema (CME), the main cause of vision drop.
   Mechanism: OCT measures retinal thickness and fluid pockets. Reducing intraretinal fluid correlates with better vision and guides need for periocular, intraocular, or systemic therapy. University of Utah Healthcare

4. Electroretinography (ERG) tracking
   Purpose: Measure whole-retina function over time.
   Mechanism: 30-Hz cone flicker timing and amplitude are sensitive markers; progressive delay or amplitude loss signals insufficient control even if the fundus looks quiet. PMC+1

5. Low-vision rehabilitation (as needed)
   Purpose: Maintain independence and quality of life if contrast sensitivity or fields decline.
   Mechanism: Optical aids, lighting optimization, orientation training, and tech adaptations reduce disability while medical therapy protects remaining function. Genetic Disease Center

6. Smoking cessation
   Purpose: Reduce uveitis risk and severity and improve vascular health.
   Mechanism: Smoking promotes systemic inflammation and is associated with higher odds of noninfectious uveitis; quitting removes this inflammatory trigger and may help treatment response. PMC+1

7. Sleep optimization
   Purpose: Support immune balance and reduce systemic inflammatory tone.
   Mechanism: Poor sleep dysregulates innate/adaptive immunity and raises inflammatory mediators; consistent sleep hygiene reduces that background “immune noise.” Nature+1

8. Regular aerobic and strength exercise
   Purpose: Lower chronic inflammation, support metabolic health, and protect microvasculature.
   Mechanism: Repeated training reduces CRP/TNF-α and improves endothelial function; better systemic control often helps ocular inflammatory diseases. PMC+1

9. Mediterranean-style diet
   Purpose: Support anti-inflammatory nutrition long-term.
   Mechanism: Diets rich in fruits/vegetables, whole grains, legumes, nuts, olive oil, and fish can lower inflammatory markers and improve endothelial function in trials. BioMed Central+1

10. Vaccination & infection prevention (per guidelines)
    Purpose: Reduce infection-triggered flares and protect when immunosuppressed.
    Mechanism: Timely non-live vaccines (per primary care) lower infectious triggers; live vaccines are coordinated carefully around immunosuppression. PMC

11. UV and glare control
    Purpose: Improve comfort and contrast, especially with photophobia.
    Mechanism: Quality sunglasses, hats, and filters cut stray light and glare that worsen symptoms while disease control proceeds. Genetic Disease Center

12. Stress management and mental-health care
    Purpose: Reduce stress-related immune activation and support adherence.
    Mechanism: Chronic inflammatory diseases carry mental-health burdens; counseling and stress-reduction can improve coping and treatment consistency. The Guardian

13. Blood pressure, blood sugar, and lipid control
    Purpose: Protect retinal microvasculature and limit edema.
    Mechanism: Systemic vascular risks worsen retinal leakage; optimizing them complements anti-inflammatory therapy. Genetic Disease Center

14. Occupational/screen ergonomics
    Purpose: Reduce eye strain and maximize functional vision during flares.
    Mechanism: Larger fonts, high-contrast themes, breaks, and lighting placement improve comfort and performance as inflammation settles. Genetic Disease Center

15. Driving and safety counseling
    Purpose: Prevent accidents if visual fields/contrast are impaired.
    Mechanism: Formal field testing guides safe driving decisions and adaptations until disease is controlled. EyeWiki

16. Home monitoring
    Purpose: Prompt detection of macular changes.
    Mechanism: Self-checks for new blur, distortion, or scotomas lead to earlier review and OCT before vision falls. University of Utah Healthcare

17. Allergy/irritant reduction
    Purpose: Avoid extra ocular surface inflammation that worsens comfort.
    Mechanism: Lubrication and trigger avoidance help dry-eye symptoms that often coexist with uveitis therapies. PMC

18. Education on medication adherence
    Purpose: Prevent relapse and macular damage.
    Mechanism: Understanding step-down plans for steroids and the slow onset of IMT/biologics improves adherence and outcomes. PMC

19. Fall-risk and home lighting optimization
    Purpose: Protect mobility if night vision is reduced.
    Mechanism: Better illumination and layout adjustments compensate for rod-function loss that ERG often shows in birdshot. PMC

20. Support groups/patient networks
    Purpose: Improve coping in a chronic, rare disease.
    Mechanism: Peer advice and reliable information (patient societies) help patients navigate long-term therapy. BUSNA

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Drug treatments

1. Prednisone (systemic corticosteroid)
   Class: Corticosteroid. Dose/Time: Often 0.5–1 mg/kg/day short-term, then slow taper; pulses (e.g., IV methylprednisolone) for severe activity. Purpose: Rapidly suppress active inflammation and macular edema. Mechanism: Broad genomic/non-genomic suppression of inflammatory cytokines and leukocyte trafficking. Side effects: Weight gain, glucose rise, hypertension, mood change, osteoporosis, cataract, glaucoma—hence the need to taper and add steroid-sparing agents. (Systemic steroid use and taper strategies in NIU are standard.) PMC+1

2. Methylprednisolone IV “pulse”
   Class: Corticosteroid. Dose/Time: 500–1000 mg IV daily for 1–3 days for sight-threatening flares, then oral taper. Purpose/Mechanism: Very rapid cytokine shutdown to protect the macula/optic nerve. Side effects: Transient insomnia, glucose spikes, infection risk. PMC

3. Adalimumab (Humira) – FDA-approved for noninfectious intermediate, posterior, and panuveitis
   Class: Anti-TNF-α monoclonal antibody. Dose/Time: 40 mg SC every 2 weeks (adult regimen). Purpose: Maintain steroid-sparing control and reduce relapses; proven in phase 3 trials (VISUAL studies). Mechanism: Neutralizes TNF-α to reduce granulocyte/lymphocyte-driven inflammation in uveitis. Side effects: Injection reactions, infection risk (TB/hepatitis screening required), rare demyelination/CHF worsening. FDA Access Data+2PMC+2

4. Infliximab (off-label in uveitis)
   Class: Anti-TNF-α monoclonal antibody (IV). Dose/Time: 5–10 mg/kg at weeks 0, 2, 6 then every 4–8 weeks; escalated for refractory disease. Purpose: Control difficult birdshot with retinal vasculitis/CME when adalimumab or antimetabolites are insufficient. Mechanism: TNF-α blockade. Side effects: Infusion reactions, infections, rare hepatosplenic T-cell lymphoma (mainly with thiopurines). FDA Access Data+1

5. Methotrexate (off-label)
   Class: Antimetabolite (dihydrofolate reductase inhibitor). Dose/Time: 15–25 mg orally or SC once weekly; folic acid added; onset 6–8 weeks. Purpose: Steroid-sparing control of NIU; not inferior to mycophenolate as first-line IMT. Mechanism: Low-dose anti-inflammatory/anti-proliferative effects on T/B cells. Side effects: Cytopenias, liver toxicity, teratogenicity; labs required. FDA Access Data+1

6. Mycophenolate mofetil (CellCept) (off-label)
   Class: Antimetabolite (IMP dehydrogenase inhibitor). Dose/Time: 1–1.5 g twice daily; onset 4–8 weeks. Purpose: Common first-line steroid-sparing therapy in NIU including birdshot. Mechanism: Blocks guanine synthesis in lymphocytes, reducing auto-immunity. Side effects: GI upset, cytopenias, infection risk; pregnancy avoidance. FDA Access Data+1

7. Azathioprine (off-label)
   Class: Antimetabolite (purine analog). Dose/Time: 1–2 mg/kg/day; TPMT activity guides dosing. Purpose: Steroid-sparing control when methotrexate or mycophenolate are unsuitable. Mechanism: Reduces lymphocyte proliferation. Side effects: Leukopenia, hepatotoxicity; infection risk. AAO Journal

8. Cyclosporine (Neoral/Sandimmune) (off-label)
   Class: Calcineurin inhibitor. Dose/Time: ~2–5 mg/kg/day divided; trough levels guide dosing. Purpose: Helpful for retinal vasculitis and when antimetabolites fail. Mechanism: Inhibits T-cell IL-2 transcription. Side effects: Hypertension, nephrotoxicity, tremor, gingival hyperplasia. FDA Access Data

9. Tacrolimus (off-label)
   Class: Calcineurin inhibitor. Dose/Time: Low oral doses individualized by levels. Purpose: Alternative to cyclosporine with similar T-cell effects. Mechanism: FKBP-calcineurin blockade. Side effects: Nephrotoxicity, neurotoxicity; monitor BP/creatinine. PMC

10. Cyclophosphamide (off-label; for severe, refractory cases)
    Class: Alkylating agent. Dose/Time: Oral daily low doses or IV pulses; limited duration. Purpose: Rescue for sight-threatening disease not controlled by safer agents. Mechanism: Cytotoxic lymphocyte suppression. Side effects: Cytopenias, infertility risk, cystitis, malignancy risk—specialist use only. PMC

11. Chlorambucil (off-label; historical use)
    Class: Alkylating agent. Dose/Time: Short courses with close counts. Purpose: Rarely used today due to toxicity; may induce long remissions in select severe cases. Mechanism/Side effects: Similar cytotoxic risks as cyclophosphamide. PMC

12. Rituximab (off-label)
    Class: Anti-CD20 monoclonal antibody. Dose/Time: RA-like regimens (e.g., 1,000 mg IV day 1 & 15, repeat by response). Purpose: For refractory cases with B-cell-driven inflammation or vasculitis. Mechanism: Depletes B cells that present antigen and produce cytokines. Side effects: Infusion reactions, hypogammaglobulinemia, infection risk. PMC

13. Tocilizumab (off-label)
    Class: IL-6 receptor blocker. Dose/Time: IV or SC per rheumatology protocols. Purpose: Rescue for persistent CME and inflammation not controlled by TNF blockade or antimetabolites. Mechanism: Dampens IL-6 signaling that drives vascular leakage. Side effects: Transaminitis, cytopenias, infection risk. Frontiers

14. Intravitreal dexamethasone implant (OZURDEX) – FDA-approved for noninfectious posterior uveitis
    Class: Corticosteroid implant (0.7 mg). Dose/Time: In-office injection; effect ~3–6 months; can repeat. Purpose: Reduce CME and intraocular inflammation while minimizing systemic side effects. Mechanism: Local steroid release into vitreous. Side effects: IOP rise, cataract acceleration; contraindicated with active ocular infection or advanced glaucoma. FDA Access Data+1

15. Intravitreal fluocinolone acetonide implant (YUTIQ) – FDA-approved for chronic NIU of the posterior segment
    Class: Corticosteroid micro-implant (0.18 mg). Dose/Time: Single injection; releases drug up to 3 years. Purpose: Long-term control and relapse prevention. Mechanism: Continuous local steroid delivery. Side effects: Cataract, elevated IOP; benefits shown vs. sham in RCTs. FDA Access Data+2FDA Access Data+2

16. Retisert (fluocinolone 0.59 mg surgical implant) – FDA-approved for NIU
    Class: Surgically sutured implant. Dose/Time: Long-acting release ~30 months. Purpose: Durable local control when frequent injections are impractical. Side effects: High rates of cataract and glaucoma surgery; specialist decision. FDA Access Data

17. Suprachoroidal triamcinolone (XIPERE) – FDA-approved for uveitic macular edema
    Class: Corticosteroid injectable suspension (40 mg/mL). Dose/Time: Office-based suprachoroidal delivery; repeat per label/response. Purpose: Target macular edema with limited anterior exposure. Mechanism: Delivers drug near choroid/retina where edema occurs. Side effects: IOP increase, cataract risk. FDA Access Data+1

18. Periocular (posterior sub-Tenon’s) triamcinolone (off-label)
    Class: Local corticosteroid injection. Dose/Time: Typically 40 mg; repeat by response. Purpose: Treat CME and posterior segment inflammation while reducing systemic exposure. Mechanism: Depot steroid near the sclera diffuses to macula. Side effects: IOP rise, cataract, rare globe perforation. PMC

19. Topical corticosteroids (adjunct)
    Class: Eye drops. Dose/Time: Frequent initial dosing then taper. Purpose: Help anterior spillover inflammation; limited in posterior disease. Side effects: IOP rise, cataract with chronic use. PMC

20. IVIG (intravenous immunoglobulin) (off-label, selected refractory cases)
    Class: Polyclonal IgG. Dose/Time: Cycles (e.g., 2 g/kg per cycle, divided). Purpose: Immune modulation when conventional IMT is contraindicated or not tolerated. Mechanism: Fc-mediated immune regulation. Side effects: Headache, thromboembolism risk, aseptic meningitis (rare). PMC

Practical note: In birdshot, long-term success usually requires steroid-sparing therapy; many patients need dual therapy or biologics. Targets include achieving inactive disease with prednisone ≤7.5 mg/day and controlling CME. retina-specialist.com

Dietary molecular supplements

1. Vitamin D – Supports immune regulation and may reduce inflammatory tone; correct deficiency per labs (often 800–2000 IU/day, individualized). Mechanism: modulates T-cells and cytokines. Office of Dietary Supplements+1

2. Omega-3 fatty acids (EPA/DHA) – Anti-inflammatory lipid mediators; typical 1–2 g/day combined EPA/DHA with meals (if not contraindicated). Mechanism: shift eicosanoid balance away from pro-inflammatory pathways. Office of Dietary Supplements

3. Lutein/Zeaxanthin – Macular carotenoids that support retinal antioxidant capacity; AREDS2 shows safety and macular benefits in AMD (not NIU-specific). Food-first or ~10 mg lutein + 2 mg zeaxanthin/day. National Eye Institute+1

4. Zinc (AREDS-style dosing only if advised) – Cofactor for antioxidant enzymes; excessive zinc can harm—use only under guidance. Mechanism: antioxidant/immune enzyme support. National Eye Institute

5. N-Acetylcysteine (NAC) – Precursor to glutathione; studied for ocular surface and anti-oxidative effects; common doses 600–1200 mg/day if appropriate. Mechanism: boosts glutathione and reduces ROS. PubMed

6. Resveratrol – Polyphenol with anti-inflammatory and neuroprotective effects in preclinical eye studies; typical supplement doses vary (100–500 mg/day), quality matters. Mechanism: antioxidant pathways (SIRT1/AMPK). PMC+1

7. Curcumin (with piperine for absorption) – Anti-inflammatory phytochemical; pilot ocular inflammation data exist but are not definitive; discuss interactions. Mechanism: NF-κB and cytokine modulation. PMC

8. Coenzyme Q10 – Mitochondrial antioxidant; may support retinal energy metabolism under oxidative stress; consult for dosing (e.g., 100–200 mg/day). Mechanism: electron transport and antioxidant activity. PMC

9. Alpha-lipoic acid – Antioxidant that recycles glutathione and vitamins C/E; typical 300–600 mg/day; may help oxidative stress pathways. PMC

10. Quercetin – Flavonoid with anti-inflammatory/antioxidant actions; limited human ocular data; consider food sources first (onions, apples). PMC

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Immunity-booster / regenerative / stem-cell” drugs

There are no FDA-approved “stem cell drugs” or regenerative cell therapies for birdshot/vitiliginous chorioretinitis. Unregulated stem-cell clinics can be dangerous and have caused severe vision loss. Instead, advanced immune-modulating therapies (some FDA-approved for NIU, some off-label) are used to safely rebalance immunity:

1. Adalimumab (anti-TNF-α, FDA-approved for NIU) – steroid-sparing control in many patients; SC every 2 weeks. FDA Access Data

2. Infliximab (anti-TNF-α, IV; off-label for NIU) – for refractory vasculitic/CME-predominant disease. FDA Access Data

3. Tocilizumab (anti-IL-6R; off-label) – helpful in stubborn CME after TNF blockers. Frontiers

4. Rituximab (anti-CD20; off-label) – B-cell depletion for select refractory cases. PMC

5. Intravitreal dexamethasone implant (OZURDEX; FDA-approved) – local, sustained steroid to reduce macular edema. FDA Access Data

6. Suprachoroidal triamcinolone (XIPERE; FDA-approved for uveitic macular edema) – targeted steroid delivery near the choroid. FDA Access Data

If you ever see “stem-cell injections for uveitis” advertised, avoid them and speak with your uveitis specialist. (Safety principle grounded in current NIU practice reviews.) PMC

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Surgeries (what they are and why they’re done)

1. Fluocinolone surgical implant (Retisert) – A small steroid device is sutured inside the eye to give ~30 months of drug for chronic posterior uveitis when frequent injections are not feasible. Done to prevent relapses and CME; risks include cataract and glaucoma surgery. FDA Access Data

2. Cataract extraction with intraocular lens – Many patients need cataract surgery because chronic inflammation and steroids cloud the lens. The goal is to restore clarity once inflammation is well controlled. PMC

3. Glaucoma surgery (trabeculectomy or tube shunt) – Performed when steroid-induced or inflammation-related eye pressure stays high despite drops, to protect the optic nerve. PMC

4. Pars plana vitrectomy – Removes dense vitreous opacities or epiretinal membranes, improves view and sometimes reduces inflammatory load; also used for diagnostic or treatment delivery reasons. PMC

5. Membrane peel (macular pucker) – If an epiretinal membrane distorts the macula and blurs vision, peeling can improve anatomy and function when the eye is quiet. PMC

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Preventions

You cannot fully “prevent” this disease, but you can prevent vision loss by reducing inflammatory triggers and acting early:

1. Don’t smoke; quitting lowers systemic inflammation linked with uveitis. PMC

2. Keep scheduled eye visits with OCT/ICGA/ERG as advised. Ajo

3. Take medicines exactly as prescribed; do not self-taper steroids. PMC

4. Screen and treat infections promptly; update vaccinations per clinician guidance. PMC

5. Maintain healthy sleep and stress management to support immune balance. Nature

6. Follow an anti-inflammatory dietary pattern (Mediterranean-style). BioMed Central

7. Exercise weekly to lower CRP/TNF-α and improve vascular health. PMC

8. Control blood pressure, glucose, and lipids with your primary care team. Genetic Disease Center

9. Use UV/glare protection and safe lighting to reduce symptoms and strain. Genetic Disease Center

10. Seek low-vision rehab early if function drops—don’t wait. Genetic Disease Center

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When to see doctors (now vs. routine)

Seek urgent care now if you notice quick vision drop, new central blur or distortion, a curtain in vision, many new floaters/flashes, severe eye pain, or sudden field loss. These can signal active inflammation, macular edema, retinal tear/detachment, or dangerous pressure rise. Keep routine visits every 1–3 months during active treatment (then less often when stable) to adjust therapy using OCT/ICGA/ERG and visual fields. Early adjustment prevents atrophy and permanent loss. Ajo+1

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What to eat and “what to avoid

Eat more:
• Colorful vegetables and fruits daily (antioxidants, carotenoids). BioMed Central
• Whole grains, legumes, nuts, and seeds for fiber and micronutrients. BioMed Central
• Fish (especially oily fish) 1–2×/week for EPA/DHA. Office of Dietary Supplements
• Olive oil as main fat; minimize trans-fats. BioMed Central
• Water and unsweetened beverages to support general health.

Limit/avoid:
• Sugary drinks and ultra-processed snacks that raise systemic inflammation. BioMed Central
• Excess alcohol (interacts with many meds, raises BP/triglycerides). PMC
• High-salt ultra-processed foods (BP/edema). BioMed Central
• Smoking/vaping and secondhand smoke (eye/immune harm). PMC
• Unverified supplements/“stem-cell” cures advertised online. PMC

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FAQs

1. Is vitiliginous chorioretinitis the same as birdshot?
   Yes—these terms describe the same disease pattern with cream, depigmented choroidal spots and strong HLA-A29 linkage. PMC

2. How rare is it?
   It’s uncommon; population estimates are low, with higher rates in HLA-A29-positive, often European-ancestry groups. Orpha

3. What causes it?
   Likely autoimmune T-cell–mediated inflammation triggered in genetically susceptible (HLA-A29) individuals. MDPI

4. Which tests are most helpful?
   ICGA for choroid, FA for vascular leakage, OCT for macular edema, ERG for function, plus HLA-A29 typing. PubMed+2Ajo+2

5. Can it burn out by itself?
   Activity may fluctuate, but uncontrolled inflammation leads to atrophy; treatment is recommended to protect vision. Nature

6. What is the main cause of vision loss?
   Cystoid macular edema and progressive outer retinal damage are key causes. University of Utah Healthcare

7. Are steroids enough?
   Short-term, yes; long-term, most patients need steroid-sparing therapy to avoid steroid side effects. PMC

8. Which biologic has the strongest evidence?
   Adalimumab is FDA-approved for NIU and supported by phase 3 trials; others are off-label but useful in refractory cases. FDA Access Data+1

9. What about implants?
   OZURDEX (dexamethasone), YUTIQ/Retisert (fluocinolone) are approved local options that reduce relapses/CME; watch for IOP rise/cataract. FDA Access Data+1

10. Will I need surgery?
    Possibly—for long-acting implants, cataracts, or glaucoma control—timed when inflammation is quiet. FDA Access Data

11. Can diet change the disease?
    Diet can’t cure it, but Mediterranean-style eating lowers systemic inflammation and supports overall vascular health. BioMed Central

12. Do supplements help?
    Only as adjuncts; correct vitamin D deficiency and consider omega-3s or macular carotenoids with clinician guidance. Office of Dietary Supplements+1

13. Is smoking really that bad for uveitis?
    Yes—smoking is linked with higher risk of noninfectious uveitis and worse outcomes; quitting helps. PMC

14. How often will I be seen?
    Often every 1–3 months during active treatment, then less often once stable; visits may increase if tests change. Ajo

15. What’s the long-term outlook?
    With modern steroid-sparing therapy and targeted biologics/implants, many patients maintain good vision, especially when CME is controlled early. ophthalmologyretina.org

Disclaimer: Each person’s journey is unique, treatment plan, life style, food habit, hormonal condition, immune system, chronic disease condition, geological location, weather and previous medical  history is also unique. So always seek the best advice from a qualified medical professional or health care provider before trying any treatments to ensure to find out the best plan for you. This guide is for general information and educational purposes only. Regular check-ups and awareness can help to manage and prevent complications associated with these diseases conditions. If you or someone are suffering from this disease condition bookmark this website or share with someone who might find it useful! Boost your knowledge and stay ahead in your health journey. We always try to ensure that the content is regularly updated to reflect the latest medical research and treatment options. Thank you for giving your valuable time to read the article.

The article is written by Team RxHarun and reviewed by the Rx Editorial Board Members

Last Updated: October 27, 2025.