Autosomal Dominant Neovascular Inflammatory Vitreoretinopathy (ADNIV)

Autosomal Dominant Neovascular Inflammatory Vitreoretinopathy (ADNIV) is a rare, inherited eye disease that runs in families in an autosomal dominant pattern. It is caused by damaging changes (mutations) in the CAPN5 gene, which encodes the protease calpain-5. These mutations appear to make calpain-5 too active (“gain-of-function”), which drives chronic eye inflammation (uveitis), new abnormal blood vessels (neovascularization), scarring membranes on the retina, fluid in the macula, and—over years—tractional retinal detachment and severe vision loss. Doctors recognize stages that start with subtle inflammation and abnormal ERG findings and may progress to neovascularization, fibrosis, glaucoma, detachment, and end-stage phthisis if untreated. Genetic testing and multimodal retinal imaging help confirm the diagnosis and guide treatment. PMC+4PMC+4PMC+4

ADNIV is a very rare, inherited eye disease that causes long-lasting inflammation inside the eye (uveitis), new abnormal blood vessels (neovascularization), and progressive damage and scarring of the retina and vitreous. It usually starts in the teens or early adulthood and gets worse over decades. Without effective control, people often lose vision in both eyes. The root cause is a harmful change (mutation) in a single gene called CAPN5, which makes an enzyme (calpain-5) too active. That overactivity sets off immune attacks in the eye, leakage and growth of new vessels, swelling, and finally scar tissue that can detach the retina. ADNIV is inherited in an autosomal dominant way, so one altered copy of the gene is enough to cause disease, though severity can vary among family members. EyeWiki+2PLOS+2

Other names

Doctors may also call this condition:

• CAPN5-associated uveitis or CAPN5-associated neovascular inflammatory vitreoretinopathy.

• Hereditary uveitis due to CAPN5.

• A uveitic masquerade syndrome, because at different times it can look like more common diseases (uveitis, retinitis pigmentosa, or proliferative diabetic retinopathy). PLOS+1

Types

ADNIV is often described in five time-based stages that each last roughly a decade, from early inflammation to late scarring and severe vision loss. Early stages show vitreous cells and electroretinogram (ERG) changes; later stages add cystoid macular edema, retinal and iris neovascularization, membranes, traction, and retinal detachment. These stages help doctors monitor progression and guide treatment intensity. EyeWiki

Causes

Important honesty in plain words: There is only one proven cause of ADNIV—pathogenic variants in the CAPN5 gene. Everything else below are mechanisms and drivers that make the disease active or worsen over time. Listing them helps readers understand why the eye becomes inflamed, forms new vessels, and scars.

1. Pathogenic CAPN5 variants (missense mutations). Multiple disease-causing changes have been identified (e.g., R243L, L244P, and others). EyeWiki+2PubMed+2

2. Gain-of-function calpain-5 activity. The mutant enzyme is overactive, needing less calcium to turn on, and cuts proteins excessively. PubMed

3. Autoimmune inflammation of the eye. T-cells infiltrate ocular tissues and sustain uveitis. EyeWiki

4. Breakdown of the blood-retina barrier, letting fluid and immune mediators leak into the retina. PLOS

5. Angiogenic signaling (e.g., VEGF upregulation) that promotes abnormal vessel growth. PLOS

6. Cystoid macular edema from chronic inflammation and leaky vessels. EyeWiki

7. Proliferative vitreoretinopathy and fibrosis (membrane formation that contracts). EyeWiki

8. Traction on the retina from contracting membranes, predisposing to detachment. PMC

9. Vitreous hemorrhage when fragile new vessels break. EyeWiki

10. Neovascularization of the iris, which can block drainage angles and raise eye pressure. EyeWiki

11. Secondary glaucoma (including neovascular glaucoma) from inflammation and new vessels. EyeWiki

12. Photoreceptor degeneration (progressive retinal cell loss). PLOS

13. Electrophysiologic dysfunction, notably early ERG b-wave loss that signals inner retinal circuit impairment. mahajanlab.stanford.edu

14. Variable expressivity and incomplete penetrance, which modulate severity within families. EyeWiki

15. De novo CAPN5 mutations, causing disease without a family history in some children. EyeWiki+1

16. Ancestry clustering (founder effect) noted in original families (e.g., northern European ancestry). EyeWiki

17. Age-related cumulative inflammation—decade-long cycles of flare and scarring. EyeWiki

18. Treatment-refractory inflammation despite systemic immunosuppression in some pediatric cases, letting disease mechanisms continue. PubMed

19. Persistent oxidative and ischemic stress in damaged retina, reinforcing neovascular loops (inferred from pathophysiology of proliferative retinopathies). PLOS

20. Genotype–phenotype diversity—distinct CAPN5 variants can track with milder vs more severe presentations. PMC

Symptoms

1. Floaters. Small moving spots or cobwebs from inflammation or bleeding in the vitreous gel. EyeWiki

2. Blurry vision. Caused by macular swelling, inflammation, or vitreous haze. EyeWiki

3. Flashes of light (photopsia). Traction on the retina can trigger light streaks. EyeWiki

4. Eye redness and irritation. Inflammation in the front chamber or throughout the eye. EyeWiki

5. Light sensitivity (photophobia). Inflamed eyes are uncomfortable in bright light. EyeWiki

6. Night-vision difficulty. Photoreceptor dysfunction and retinal degeneration reduce dark adaptation. PLOS

7. Peripheral vision loss. Progressive retinal damage and scarring narrow the field. PMC

8. Distortion of straight lines (metamorphopsia). Macular edema or traction warps central vision. EyeWiki

9. Episodes of sudden blur from vitreous hemorrhage. EyeWiki

10. Seeing shadows/curtains. A warning sign of retinal detachment. PMC

11. Headaches or eye pain if pressure rises from neovascular or inflammatory glaucoma. EyeWiki

12. Poor contrast sensitivity. Photoreceptor and inner retinal dysfunction reduce fine detail. mahajanlab.stanford.edu

13. Color vision changes with macular or photoreceptor damage. PLOS

14. Fluctuating vision as inflammation and edema wax and wane. EyeWiki

15. Severe, long-term vision loss in advanced stages, sometimes to no light perception. PMC

Diagnostic tests

A) History & physical/clinical examination (eye-focused physical exam)

1. Best-corrected visual acuity (chart testing). Tracks how clearly you can see; worsening acuity signals active swelling, bleeding, or scarring. Also forms the baseline for judging treatment response. EyeWiki

2. Intraocular pressure (IOP) measurement. Detects steroid-related rises or neovascular glaucoma—both need prompt treatment. EyeWiki

3. Slit-lamp biomicroscopy. Looks for anterior chamber cells/flare, keratic precipitates, iris neovascularization, and lens changes; documents active uveitis. EyeWiki

4. Dilated fundus examination (indirect ophthalmoscopy). Directly visualizes vitreous cells, hemorrhage, retinal neovascularization, membranes, traction, and detachment. EyeWiki

5. Gonioscopy. Checks the drainage angle for abnormal iris vessels that can cause pressure spikes. EyeWiki

B) Office “manual/functional” tests

6. Amsler grid. Simple at-home/office grid detects central distortion from macular edema. EyeWiki

7. Confrontation visual fields or perimetry (e.g., Goldmann). Maps peripheral field loss from retinal degeneration and scarring. PMC

8. Color vision testing (Ishihara or similar). Identifies macular/photoreceptor dysfunction affecting color discrimination. PLOS

C) Laboratory & pathology

9. Genetic testing for CAPN5 variants. Confirms the diagnosis, enables family screening, and can prompt earlier surveillance in at-risk relatives. PLOS+1

10. General uveitis work-up to exclude mimics (targeted tests guided by history: e.g., syphilis serology, TB testing, ACE for sarcoid, autoimmune panels). ADNIV is genetic, but ruling out other causes is standard when presentation is unclear. EyeWiki

11. Inflammatory markers (ESR/CRP)—often nonspecific, but may help document systemic inflammation or exclude other entities in atypical cases. EyeWiki

12. Aqueous/vitreous sampling (research/selected cases). Cytokine or cell analysis can characterize inflammation when the diagnosis is uncertain; rarely needed once CAPN5 is identified. EyeWiki

D) Electrodiagnostic testing

13. Full-field ERG. A hallmark of ADNIV is early loss of the b-wave, signaling inner retinal dysfunction before advanced structural damage is obvious. Serial ERGs track progression. mahajanlab.stanford.edu

14. Pattern ERG or multifocal ERG. Adds detail about macular and ganglion-cell level function in chronic disease. mahajanlab.stanford.edu

15. EOG (electro-oculography). Assesses retinal pigment epithelium function, sometimes altered in pigmentary degeneration. PLOS

16. VEP (visual evoked potentials). Confirms downstream visual pathway function when acuity or fields are limited by media opacity. PLOS

E) Imaging

17. Optical coherence tomography (OCT). Shows cystoid macular edema, epiretinal membranes, vitreomacular traction, and overall retinal layer damage; crucial for treatment decisions. EyeWiki

18. OCT angiography (OCTA). Non-invasive view of capillary dropout and neovascular complexes in the macula and periphery. ophthalmologyretina.org

19. Fluorescein angiography (FA). Maps leakage, ischemia, and neovascularization across the retina; often done with ultra-widefield systems in ADNIV. ophthalmologyretina.org

20. B-scan ultrasonography. Useful when vitreous hemorrhage or cataract prevents fundus view; detects traction and detachment. EyeWiki

Non-pharmacological treatments (therapies & others)

Below are practical, clinic-ready options. Each paragraph explains what, purpose, and mechanism in simple English.

1. Regular dilated eye exams with imaging — Purpose: catch silent changes early. Mechanism: widefield photos, fluorescein angiography, and OCT show leakage, neovascularization, fibrosis, and macular edema so therapy can be timed before complications. EyeWiki+1

2. Genetic counseling & testing for CAPN5 — Purpose: confirm diagnosis, inform family screening. Mechanism: finding a CAPN5 mutation that increases protease activity supports the diagnosis and helps relatives get checked sooner. PMC+1

3. Treat systemic infectious/inflammatory mimics first — Purpose: exclude infections and systemic causes of uveitis. Mechanism: lab workup and targeted therapy prevent inappropriate immunosuppression. (General uveitis management guidance.) AAO+1

4. Protective eyewear / trauma avoidance — Purpose: reduce risk of retinal tears/bleeds in fragile retinas. Mechanism: prevents trauma-related breaks that can precipitate detachment. (General detachment risk counseling.) EyeWiki

5. Tight control of comorbid diabetes or hypertension (if present) — Purpose: minimize additive vascular stress. Mechanism: high glucose and blood pressure worsen retinal ischemia and neovascular drive; standard PDR care shows benefit of vascular risk control. AAO

6. Smoking cessation — Purpose: protect retinal microvasculature and inflammation balance. Mechanism: smoking worsens retinal vascular disease and reduces ocular perfusion; cessation is standard in retinal disease counseling. AAO

7. Panretinal photocoagulation (PRP) when neovascularization threatens — Purpose: reduce ischemic VEGF drive and prevent bleeds/detachments. Mechanism: laser ablates ischemic retina to lower VEGF, a main signal for new vessels; established for PDR and extrapolated in ADNIV when neovascularization appears. AAO+1

8. Focal/grid laser for selected leakage — Purpose: reduce focal edema from leaky microvasculature. Mechanism: photocoagulation seals focal leakage; used selectively alongside pharmacotherapy. AAO Journal

9. Vitreous hemorrhage precautions (head elevation, avoid blood thinners unless essential) — Purpose: limit prolonged visual obscuration. Mechanism: gravity aids settling; avoiding non-essential antiplatelet/NSAID use reduces recurrent bleeding risk (decision with PCP). (General retinal care principles.) EyeWiki

10. Cycloplegia for comfort during flares — Purpose: reduce pain, photophobia, and prevent posterior synechiae. Mechanism: mydriatic/cycloplegic drops relax ciliary muscle and keep the iris moving. EyeWiki

11. Intraocular pressure (IOP) monitoring — Purpose: detect steroid response or neovascular/angle-closure glaucoma early. Mechanism: routine IOP checks catch pressure rises requiring therapy. webeye.ophth.uiowa.edu

12. Early referral to a uveitis/retina specialist — Purpose: coordinate steroids, immunomodulators, anti-VEGF, and surgery at the right times. Mechanism: complex staging and tailored plans improve outcomes in series of ADNIV patients. PubMed+1

13. Patient education on red-flag symptoms — Purpose: prompt care for new floaters, curtain, sudden blur, or pain. Mechanism: earlier detachment/bleed treatment preserves vision. (Standard retinal detachment education.) AAO

14. Treat ocular surface inflammation/dryness — Purpose: keep the front of the eye comfortable and reduce confounders. Mechanism: lubricants and lid hygiene improve surface stability; helpful alongside uveitis care. (General uveitis practice.) AAO

15. Systemic health optimization (sleep, diet quality, exercise) — Purpose: support immune balance and vascular health. Mechanism: cardiometabolic fitness lowers systemic inflammatory tone that can amplify ocular disease. (General medical guidance embedded in AAO care frameworks.) AAO

16. Careful steroid stewardship — Purpose: use enough to control inflammation but avoid long-term complications. Mechanism: taper plans and switch to steroid-sparing agents when possible per uveitis best practice. AAO+1

17. Prompt management of cystoid macular edema (CME) — Purpose: preserve central vision. Mechanism: OCT monitoring; treat with peri/intravitreal steroid or systemic/steroid-sparing agents as indicated. PMC

18. Anticoagulation review — Purpose: avoid unnecessary bleeding risk in eyes with fragile neovascularization. Mechanism: coordinate with cardiology/medicine to keep essential meds but minimize extras. (General retinal surgery/bleed risk principles.) AAO Journal

19. Pre-op planning for cataract in inflamed eyes — Purpose: reduce flares and macular edema after surgery. Mechanism: peri-operative anti-inflammatory control per uveitis surgery guidance. AAO

20. Timely vitrectomy when indicated — Purpose: remove hemorrhage, membranes, and relieve traction. Mechanism: surgery clears media, peels fibrotic tissue, and repairs detachment to preserve structure. (ADNIV series and standard tractional detachment care.) PMC+1

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Drug treatments

Important: Except for adalimumab (approved for non-infectious uveitis) and intravitreal steroid implants (approved for posterior uveitis), none are FDA-approved for ADNIV. Doses below summarize FDA-labeled regimens for their approved indications; retina/uveitis specialists tailor regimens for ADNIV off-label.

1. Adalimumab (Humira / biosimilars such as Amjevita, Hulio) — Class: anti-TNF-α. Dose/time: Adults with non-infectious uveitis: 80 mg once, then 40 mg every other week starting one week later (subcutaneous). Purpose: control non-infectious intermediate/posterior uveitis. Mechanism: neutralizes TNF-α to reduce ocular inflammation. Side effects: serious infection risk, TB reactivation, malignancy warnings. FDA label cited. FDA Access Data+2FDA Access Data+2

2. Dexamethasone intravitreal implant (Ozurdex 0.7 mg) — Class: corticosteroid. Indication: non-infectious posterior uveitis (also RVO/DME). Purpose/mechanism: sustained steroid reduces vascular leakage and cytokine signaling. Notes: watch IOP and cataract risk. FDA label cited. FDA Access Data+1

3. Fluocinolone acetonide intravitreal implant (Yutiq 0.18 mg) — Class: corticosteroid implant. Indication: chronic non-infectious posterior uveitis. Purpose/mechanism: multi-year low-dose steroid release to suppress relapses. Side effects: cataract, IOP rise. FDA review/label cited. FDA Access Data+1

4. Fluocinolone acetonide implant (Iluvien 0.19 mg) — Class: corticosteroid (indicated for DME). Purpose in practice: off-label steroid control in refractory posterior uveitis where long duration is desired. Mechanism/risks: very long-acting steroid; cataract/IOP. FDA label cited. FDA Access Data

5. Aflibercept (Eylea/Eylea HD) intravitreal — Class: anti-VEGF decoy receptor. Indications: AMD, RVO, DME. Purpose in ADNIV: off-label suppression of neovascularization and CME when ischemic drive is present. Mechanism: binds VEGF-A/PlGF to reduce leakage and new vessels. Key risks: endophthalmitis, intraocular inflammation. FDA label cited. FDA Access Data+1

6. Ranibizumab (Lucentis) intravitreal — Class: anti-VEGF antibody fragment. Indications: AMD, RVO, DME, myopic CNV. Purpose in ADNIV: off-label for uveitic CNV or edema. Mechanism/risks: neutralizes VEGF-A; infection/inflammation precautions. FDA label cited. FDA Access Data+1

7. Topical difluprednate (Durezol 0.05%) — Class: potent topical corticosteroid. Indications: postoperative inflammation, endogenous anterior uveitis. Purpose in ADNIV: control anterior segment flares that accompany posterior disease. Mechanism/risks: steroid; monitor IOP. FDA label cited. FDA Access Data

8. Topical prednisolone acetate 1% (Pred Forte/Omnipred) — Class: topical corticosteroid. Indication: steroid-responsive ocular inflammation. Purpose: adjunct for anterior chamber cells/flare. Mechanism/risks: steroid; IOP/cataract risk. FDA labels cited. FDA Access Data+1

9. Methotrexate (systemic; periocular/intravitreal in specialist hands) — Class: antimetabolite immunomodulator. Purpose in ADNIV: steroid-sparing control of chronic non-infectious uveitis. Mechanism: inhibits folate pathways to damp adaptive immunity. Risks: bone-marrow, hepatic, teratogenic. FDA label cited (systemic dosing/risks). FDA Access Data

10. Mycophenolate mofetil (CellCept) — Class: antimetabolite immunosuppressant. Purpose: steroid-sparing in chronic uveitis. Mechanism: inhibits inosine monophosphate dehydrogenase, suppressing lymphocyte proliferation. Risks: infection, GI, teratogenic. FDA label cited. FDA Access Data

11. Azathioprine (Imuran) — Class: purine antimetabolite. Purpose: alternate steroid-sparing agent. Mechanism: inhibits DNA synthesis in rapidly dividing immune cells. Risks: myelosuppression (TPMT testing), malignancy warning. FDA label cited. FDA Access Data+1

12. Cyclosporine (Neoral/Sandimmune) — Class: calcineurin inhibitor. Purpose: T-cell suppression in refractory uveitis. Mechanism: blocks IL-2 transcription; reduces T-cell activation. Risks: nephrotoxicity, hypertension, drug interactions. FDA labels cited. FDA Access Data+1

13. Tacrolimus (Prograf) — Class: calcineurin inhibitor. Purpose: alternative T-cell targeted immunosuppression. Mechanism: FKBP-12 complex inhibits calcineurin; lowers IL-2. Risks: nephrotoxicity, neurotoxicity, infection. FDA label cited. FDA Access Data

14. Acetazolamide (Diamox) — Class: carbonic anhydrase inhibitor. Purpose: adjunct to reduce cystoid macular edema in some inflammatory retinopathies. Mechanism: reduces fluid accumulation by altering retinal pigment epithelium fluid transport. Risks: paresthesia, renal stone risk, sulfa allergy. FDA label cited (systemic CAI information). FDA Access Data

15. Periocular/intravitreal triamcinolone acetonide — Class: corticosteroid. Purpose: short-to-intermediate control of edema/inflammation when implants aren’t used. Mechanism/risks: steroid with IOP/cataract risk. (Use consistent with steroid class labeling; specific branded labels vary.) PMC

16. Systemic corticosteroids (short courses) — Class: glucocorticoid. Purpose: rapidly quiet severe flares or vision-threatening inflammation before steroid-sparing therapy takes effect. Mechanism: broad anti-inflammatory gene regulation; taper to avoid relapse. Representative label risks noted (cataract/IOP from steroid class). FDA Access Data

17. Anti-VEGF plus steroid “combo” protocols — Purpose: target both leakage (VEGF) and inflammation (cytokines) when neovascularization/CME coexist. Mechanism: dual-pathway suppression; used in ADNIV case reports/series. Risks: see respective labels. IOVS+1

18. IOP-lowering drops (if steroid or neovascular glaucoma occurs) — Purpose: protect optic nerve. Mechanism: aqueous suppression or outflow enhancement per glaucoma practice; selection individualized. (Pharmacologic classes per label; agent choice varies.) AAO

19. Antibiotic-steroid combo drops (short course if surface infection risk) — Purpose: treat concurrent surface infection while controlling inflammation on the surface only (not for posterior disease). Mechanism: antimicrobial plus steroid. Risks: steroid masking; specialist guidance needed. FDA example label cited. FDA Access Data

20. Vaccination/TB/viral screening before biologics — Purpose: reduce serious infection risk when using adalimumab or other potent immunosuppressants. Mechanism: follow biologic label warnings and uveitis guidance. FDA biologic labels & AAO guidance. FDA Access Data+1

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Dietary molecular supplements

No supplement has been proven to treat ADNIV. Some nutrients support general retinal health, mainly studied in AMD (AREDS/AREDS2); benefits do not automatically extend to ADNIV. Discuss with your ophthalmologist before starting anything.

1. AREDS2-style antioxidant mix (vit C, vit E, zinc, copper, lutein/zeaxanthin) — Purpose: overall retinal antioxidant support. Mechanism: lowers oxidative stress; AREDS2 supports benefit in intermediate AMD, not ADNIV. Dose patterns mirror AREDS2 formulations. National Eye Institute+1

2. Lutein — Purpose: macular pigment support. Mechanism: filters blue light/antioxidant; studied in AMD. (Typical doses in AREDS2: 10 mg/d with zeaxanthin.) National Eye Institute

3. Zeaxanthin — Purpose/mechanism: as above (2 mg/d in AREDS2 formula). National Eye Institute

4. Omega-3 fatty acids (DHA/EPA) — Purpose: anti-inflammatory membrane effects; no added benefit to AREDS formula in AREDS2. Use as part of a healthy diet (fatty fish). National Eye Institute

5. Zinc (with copper) — Purpose: antioxidant enzyme cofactor. Mechanism: supports retinal metabolism; use balanced with copper to avoid deficiency. National Eye Institute

6. Vitamin C — Purpose: antioxidant. Mechanism: scavenges reactive oxygen species; best obtained in diet. National Eye Institute

7. Vitamin E — Purpose: lipid antioxidant; role in AMD mixed; avoid high doses without supervision. National Eye Institute

8. Carotenoid-rich foods (spinach, kale, corn) — Purpose: dietary sources of lutein/zeaxanthin rather than pills. Mechanism: accumulates in macular pigment. National Eye Institute

9. Balanced Mediterranean-style diet — Purpose: vascular/anti-inflammatory benefits; good for retina as extrapolated from diabetic retinopathy/AMD care. Mechanism: emphasizes plants, whole grains, fish, olive oil. AAO

10. Hydration & salt moderation — Purpose: general vascular health; helps BP control which affects retinal perfusion. Mechanism: supports systemic homeostasis. AAO

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Immunity-booster / regenerative / stem-cell” drugs

There are no FDA-approved “immunity-booster” or “stem-cell drugs” for ADNIV. Approved options relevant to non-infectious uveitis are anti-TNF-α (adalimumab) and steroid implants listed above. “Stem cell” eye injections sold in clinics are often unapproved and risky; patients have been harmed. Below are safe, FDA-approved categories actually used to regulate immunity in uveitis (not to “boost” it), or to provide durable steroid effect.

1. Adalimumab products (Humira/Amjevita/Hulio) — Immune-modulating biologic (not a booster) approved for non-infectious uveitis; dosing/risks per label above. FDA Access Data+2FDA Access Data+2

2. Dexamethasone intravitreal implant (Ozurdex) — durable intraocular steroid for posterior uveitis. FDA Access Data

3. Fluocinolone acetonide implant (Yutiq) — very long-acting implant for chronic posterior uveitis. FDA Access Data

4. Cyclosporine (systemic) — calcineurin inhibitor used off-label as a steroid-sparing agent in uveitis; immune suppression per FDA label. FDA Access Data

5. Tacrolimus (systemic) — calcineurin inhibitor used off-label in refractory uveitis; immune suppression per FDA label. FDA Access Data

6. Methotrexate (systemic) — antimetabolite used off-label in uveitis; immune modulation per FDA label. FDA Access Data

(If you see offers of “stem-cell cures” for inherited retinal disease outside clinical trials, treat them as unsafe and unapproved.)

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Surgeries

1. Pars plana vitrectomy with membrane peeling — Removes vitreous hemorrhage and fibrotic epiretinal membranes that pull on the retina; reduces traction and allows laser/medication to work better. Indicated for non-clearing hemorrhage or traction. PMC+1

2. Retinal detachment repair (tractional) — Uses vitrectomy, membrane dissection, laser, and tamponade to re-attach the retina when fibrous bands detach it. Prevents permanent vision loss. NCBI

3. Panretinal photocoagulation (PRP) surgery — Office-based laser to treat proliferative neovascularization; prevents vitreous hemorrhage and traction. AAO

4. Glaucoma procedures (e.g., tube shunt) for neovascular or steroid-induced glaucoma — Lowers eye pressure when drops fail; protects optic nerve. AAO

5. Cataract surgery with uveitis protocol — Removes steroid/inflammation-related cataracts using peri-operative anti-inflammatory optimization to avoid CME flare. Restores clarity and visual potential. AAO

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Preventions

1. Don’t smoke; support vascular health. AAO

2. Control BP, glucose, and lipids if present. AAO

3. Keep all retina/uveitis follow-ups even when vision feels “okay.” EyeWiki

4. Eye protection for sports/impact activities. EyeWiki

5. Get vaccinations and TB/viral screening before biologics. FDA Access Data

6. Learn and act on red-flag symptoms (curtain, sudden floaters, pain). AAO

7. Avoid unnecessary blood-thinning meds without medical need. AAO

8. Maintain a Mediterranean-style diet and regular exercise. AAO

9. Use steroid drops/implants exactly as prescribed and attend IOP checks. FDA Access Data

10. Involve family—autosomal dominant inheritance means relatives may benefit from evaluation. PMC

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When to see a doctor urgently

Seek same-day care for any sudden curtain or shadow, a shower of new floaters, flashes, rapidly worsening blur, eye pain, redness, or headache with halos (possible high IOP). These symptoms can signal bleed, tear, or detachment where time matters. AAO

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What to eat and what to avoid

Eat: colorful vegetables and leafy greens (lutein/zeaxanthin sources), fruit, legumes, whole grains, nuts, fish (omega-3s), and olive oil—this supports vascular/anti-inflammatory balance seen in retinal care. Avoid/limit: tobacco; excess sodium, added sugars, and ultra-processed foods that worsen BP/glucose control; and megadose supplements not prescribed by your clinician. Remember: AREDS2-type supplements do not treat ADNIV; ask your doctor before using them. National Eye Institute+1

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FAQs

1) Is there a cure?
No cure yet. Treatment manages inflammation, leakage, and scarring to slow damage and protect vision as long as possible. Research on CAPN5 biology is ongoing. PMC

2) What gene is involved?
CAPN5 (calpain-5). Mutations make the enzyme too active, driving inflammation and damage in the retina. PMC+1

3) How is ADNIV diagnosed?
By history, examination, multimodal imaging, ERG, and genetic testing for CAPN5 mutations. EyeWiki

4) Do anti-VEGF eye injections help?
They can suppress abnormal new vessels and edema in ADNIV by analogy to proliferative retinopathies; this use is off-label but supported by case experience. IOVS+1

5) Which drug is FDA-approved for uveitis?
Adalimumab (and its biosimilars) are FDA-approved for non-infectious uveitis; Ozurdex and Yutiq are FDA-approved steroid implants for posterior uveitis. None are approved specifically for ADNIV. FDA Access Data+2FDA Access Data+2

6) Why does my doctor suggest PRP laser?
To reduce VEGF from ischemic retina, lowering the risk of bleeds and traction. AAO

7) Do supplements fix ADNIV?
No. AREDS2 nutrients help certain AMD patients; there’s no proof they treat ADNIV. Focus on diet quality and medical therapy. National Eye Institute

8) What raises eye pressure (IOP) in ADNIV?
Steroids can raise IOP; neovascularization can also cause angle-closure glaucoma. Regular checks are essential. FDA Access Data

9) Is surgery common?
Yes, when hemorrhage, membranes, or detachment threaten vision. Timing depends on imaging and exam. PMC

10) Can family members be tested?
Yes. ADNIV is autosomal dominant; first-degree relatives can consider genetic counseling/testing. PMC

11) How long will I need treatment?
ADNIV evolves over years; care is long-term with phases of quiet and flare. Plans change with stage and response. JAMA Network

12) Are “stem-cell eye injections” safe?
Unapproved stem-cell products outside trials can be dangerous. Stick to FDA-approved therapies and clinical trials. AAO

13) Why combine steroid with anti-VEGF?
ADNIV involves both inflammation and VEGF-driven leakage; dual targeting can help when edema and neovascularization coexist. Retina Specialist

14) What monitoring do I need?
Scheduled OCT/angiography, pressure checks, and symptom education—because structure can change before vision does. EyeWiki

15) What research directions exist?
CAPN5 structure-function studies and model systems aim to find targeted inhibitors for the overactive protease. Iowa Research Online

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 03, 2025.

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