CAPN5 Vitreoretinopathy

CAPN5 vitreoretinopathy is a rare, inherited eye disease caused by harmful changes (variants) in the CAPN5 gene. The disease follows an autosomal dominant pattern—one altered copy of the gene is enough to cause the condition. It starts with eye inflammation that looks like uveitis, then behaves like a retinal degeneration (similar to retinitis pigmentosa), and later adds abnormal new blood vessels, scarring, and traction that resemble advanced diabetic eye disease. Over years, this step-by-step process can lead to very poor vision if not recognized and managed early. The CAPN5 gene makes calpain-5, a calcium-activated enzyme; disease-causing variants make this enzyme overactive (gain-of-function), which disrupts normal retinal cell signaling, survival, and blood-vessel control. EyeWiki+3PubMed+3PLOS+3

CAPN5 vitreoretinopathy is a rare, inherited eye disease that slowly damages the retina (the light-sensing layer at the back of the eye). It is caused by harmful changes (mutations) in a gene called CAPN5, which makes an enzyme (calpain-5) involved in cell signaling. The disease usually runs in families in an autosomal dominant pattern, so a parent with the disease has a 1 in 2 chance of passing it on to each child. The illness typically goes through stages: early inflammation inside the eye (uveitis), then abnormal new blood vessels in the retina (neovascularization), bleeding, scar tissue and traction, and finally retinal degeneration like retinitis pigmentosa. Without treatment, vision may slowly worsen over years. There is no single “cure,” but careful monitoring and stage-based treatments can help control inflammation, bleeding, swelling, and scarring to preserve sight as long as possible. PMC

Scientists first linked CAPN5 variants to this condition in 2012 families, showing that patients experience a sequence of phases: early autoimmune uveitis, later retinal neovascularization and scarring, and progressive photoreceptor loss. Animal and cellular models show the mutant enzyme becomes active at lower calcium levels, explaining the chronic overactivity and damage. PubMed+2PMC+2

Other names

• Autosomal Dominant Neovascular Inflammatory Vitreoretinopathy (ADNIV)

• CAPN5-related vitreoretinopathy

• Neovascular inflammatory vitreoretinopathy (NIV)
  All refer to the same CAPN5-driven disorder spectrum. National Organization for Rare Disorders+1

Types

Doctors often describe stages that reflect how the disease behaves over time. You may see:

1. Early/Stage I: mild inflammatory signs in the vitreous (“cells”), often with an abnormal electroretinogram (ERG) before major vision loss. mahajanlab.stanford.edu

2. Intermediate/Stages II–III: more obvious posterior uveitis, cystoid macular edema, beginning fibrosis, and early neovascularization. mahajanlab.stanford.edu

3. Late/Stages IV–V: extensive scarring (epiretinal membranes), tractional retinal detachments, neovascular glaucoma, and progressive retinal degeneration. WebEye+1

Genetically, different CAPN5 variants can cause milder or more severe courses; some newly reported changes are linked to mild or later-onset presentations. PMC+1

---

Causes

1. CAPN5 gene variant (root cause). A single pathogenic CAPN5 change is sufficient to cause the disease (autosomal dominant). PubMed

2. Gain-of-function of calpain-5. The mutant enzyme becomes hyperactive, damaging retinal cells. PubMed+1

3. Lower calcium activation threshold. Mutant calpain-5 turns on too easily, staying active longer. EyeWiki

4. Photoreceptor stress and death. Overactive calpain-5 promotes photoreceptor degeneration. PubMed

5. Autoimmune-like eye inflammation. The retina shows chronic uveitis features. PubMed

6. Abnormal synapse signaling. Studies suggest synaptic dysfunction in the outer retina. EyeWiki

7. Pro-angiogenic drive (new vessels). Retinal ischemia/inflammation promote neovascularization. MalaCards

8. Fibrosis and membrane formation. Aberrant wound-healing causes epiretinal membranes and scarring. WebEye

9. Tractional forces. Scar tissue contracts and pulls on retina → detachments. MalaCards

10. Macular edema. Inflammatory leakage causes cystoid macular edema. PreventionGenetics

11. Vitreous hemorrhage. Fragile new vessels bleed into the eye. PreventionGenetics

12. Neovascular glaucoma. New vessels grow on the iris/angle, raising eye pressure. PreventionGenetics

13. Genotype-phenotype variation. Some variants (e.g., p.L244P, p.R243L) track with severity. Taylor & Francis Online+1

14. Defective membrane association of mutants. Many CAPN5 mutants show impaired membrane interactions, altering activity. ScienceDirect

15. Cascade amplification. Chronic enzyme overactivity may drive multiple downstream injury pathways. EyeWiki

16. Retinal ischemia. Capillary closure and nonperfusion feed into neovascularization. Ophthalmology Retina

17. Immune dysregulation. The phenotype includes features of autoimmune uveitis. PubMed

18. Extraretinal factors likely minimal. This is a primarily monogenic retinal process; systemic causes are usually not required. PubMed+1

19. Age-related expression. Many patients present in teens–30s as the mutation’s effects accumulate. PreventionGenetics

20. Modifier genes (possible). Ongoing work suggests other variants may modulate features, but CAPN5 is central. PMC

---

Common symptoms & signs

1. Floaters. Small moving spots from vitreous cells/bleeding early in disease. WebEye

2. Blurry vision. From inflammation, macular edema, or media haze. PreventionGenetics

3. Light sensitivity (photophobia). Active uveitis makes eyes light-sensitive. WebEye

4. Red or uncomfortable eye. Inflammation can cause redness and ache. WebEye

5. Night-vision trouble. Early photoreceptor dysfunction shows up in low light and on ERG. mahajanlab.stanford.edu

6. Peripheral vision loss. Ischemia, scarring, and detachments narrow side vision. MalaCards

7. Distorted central vision. Macular edema and membranes warp straight lines. PreventionGenetics

8. Episodes of vision “haze.” Vitreous cells/hemorrhage cloud the view. PreventionGenetics

9. New vessels on iris or retina. May be silent until bleeding or glaucoma occurs. WebEye

10. Increase in eye pressure. Neovascular glaucoma can raise IOP and cause pain. PreventionGenetics

11. Flashes or curtain-like shadow. These suggest tractional detachment risk. MalaCards

12. Color vision decline. Photoreceptor dysfunction reduces color discrimination. PubMed

13. Cataract formation. Chronic inflammation and steroids increase cataract risk. mahajanlab.stanford.edu

14. Slow, stepwise worsening. Disease moves through phases over years. PubMed

15. Asymmetry between eyes. One eye can advance faster than the other. PubMed

---

Diagnostic tests

A) Physical exam / clinical chair-side assessments

1. Best-corrected visual acuity. Measures central vision to track change over time. Decline may reflect macular edema, scarring, or photoreceptor loss. PreventionGenetics

2. Pupil exam. A relative afferent pupillary defect can appear with significant retinal damage. WebEye

3. Slit-lamp exam (anterior segment). Looks for cells/flare, iris neovascularization, and cataract. Key for detecting active uveitis and NVI. WebEye

4. Dilated fundus exam. Direct view of vitreous cells, retinal hemorrhages, exudates, membranes, and detachments. WebEye

B) Manual or office-based functional tests

5. Amsler grid. Quick screen for macular distortion from edema or membranes. PreventionGenetics

6. Confrontation visual fields. Bedside check for peripheral field loss; formal fields often follow. MalaCards

7. Intraocular pressure measurement (tonometry). Vital for spotting neovascular glaucoma early. PreventionGenetics

8. Color vision testing. Sensitive to photoreceptor dysfunction in progressive disease. PubMed

C) Laboratory & pathological / genetic

9. Targeted CAPN5 genetic testing. Confirms the diagnosis, enables family testing, and helps anticipate course (some variants milder/severe). PreventionGenetics+1

10. Broader retinal dystrophy panels. If CAPN5 is suspected but not obvious, panels can detect CAPN5 and rule out mimics. GeneCards

11. Baseline uveitis labs (as needed). To exclude other inflammatory causes when the picture is unclear (ADNIV itself is monogenic). PubMed

12. Pathology of membranes (rarely needed). If surgery is done, tissue may show fibrovascular proliferation consistent with proliferative stages. WebEye

D) Electrodiagnostic

13. Full-field ERG. Very sensitive; early cases can show reduced or “negative” b-wave before obvious fundus changes—helps stage and track disease. mahajanlab.stanford.edu

14. Multifocal ERG (mfERG). Maps cone system function across the macula to monitor progression. PMC

15. Electro-oculography (EOG). Assesses retinal pigment epithelium function; can support diffuse retinal dysfunction. PMC

E) Imaging

16. Optical coherence tomography (OCT). Noninvasive layers-scan that shows cystoid macular edema, epiretinal membranes, and traction. Ophthalmology Retina

17. Ultra-widefield fluorescein angiography (UWF-FA). Maps ischemia, leakage, and peripheral neovascularization—key for treatment planning. Ophthalmology Retina

18. Fundus photography / ultra-widefield color. Baseline documentation of pigment changes, hemorrhage, and fibrosis. WebEye

19. OCT-angiography (OCT-A). Non-dye view of retinal/choriocapillaris flow; can detect neovascular complexes and capillary loss. Ophthalmology Retina

20. B-scan ocular ultrasound. Useful when media are opaque (dense hemorrhage/cataract) to evaluate traction and detachments. WebEye

Non-pharmacological treatments (therapies & others)

1. Regular retina follow-up: Long-term, scheduled visits catch edema, bleeding, or pressure rises early, limiting permanent damage by allowing timely injections, laser, or surgery. Purpose: prevent avoidable vision loss. Mechanism: surveillance and prompt intervention. PMC

2. Protective UV-blocking eyewear: Reduces photic discomfort during uveitis flares and may limit light-triggered symptoms. Purpose: comfort and glare control. Mechanism: blocks UV/blue light reaching inflamed tissues. EyeWiki

3. Smoking cessation: Smoking worsens vascular and inflammatory eye disease; stopping supports retinal and overall ocular health. Purpose: reduce ischemia and inflammation risks. Mechanism: improves microvascular function and reduces oxidative stress. (General ophthalmic evidence.) EyeWiki

4. Tear and lid hygiene during flares: Gentle care lowers surface irritation that can worsen photophobia. Purpose: symptom relief. Mechanism: reduces surface inflammation that amplifies uveitic discomfort. EyeWiki

5. Low-vision rehabilitation: Magnifiers, contrast tools, and lighting strategies keep people independent as central vision fluctuates. Purpose: function and quality of life. Mechanism: optical aids + training. PMC

6. Blue-light filtering and glare control indoors: Filters and task lighting help macular edema patients read more comfortably. Purpose: reduce glare disability. Mechanism: modifies light spectrum and luminance. Ophthalmology Retina

7. Exercise within medical advice: Supports vascular health and mood during chronic disease and immunosuppression. Purpose: systemic resilience. Mechanism: anti-inflammatory myokines, improved perfusion. (General.) FDA Access Data

8. Vaccination update before systemic immunosuppression: Prevents avoidable infections during steroid/biologic therapy. Purpose: safety. Mechanism: primed immunity with inactivated vaccines (no live vaccines on biologics). (General immunosuppression guidance from biologic labels.) FDA Access Data

9. Home Amsler monitoring: Early self-detection of macular changes between visits prompts earlier care. Purpose: earlier detection. Mechanism: daily grid check. Ophthalmology Retina

10. Protective measures to avoid eye trauma: Scarred, neovascular retinas bleed easily; minimizing trauma lowers hemorrhage risk. Purpose: safety. Mechanism: reduces traction/bleed triggers. WebEye

11. Blood pressure and glucose control: Better vessel health can limit edema and complications. Purpose: reduce leakage burden. Mechanism: stabilizes endothelial permeability. (General retinal disease evidence.) Ophthalmology Retina

12. Photoprotection outdoors (hat + wrap sunglasses): Extra help for light sensitivity and surface comfort. Purpose: comfort. Mechanism: reduces scatter and UV. EyeWiki

13. Education on steroid response: Some patients’ eye pressure rises on steroids—knowing warning signs leads to faster care. Purpose: prevent glaucomatous damage. Mechanism: early IOP checks. FDA Access Data

14. Stress and sleep hygiene: Helps reduce flare-perception and improves adherence. Purpose: coping. Mechanism: autonomic balance may modulate inflammatory symptoms. (General.) PMC

15. Assistive tech (screen readers, high-contrast settings): Keeps reading/work possible during flares. Purpose: function. Mechanism: boosts contrast/enlargement. PMC

16. Nutritional pattern (leafy greens, fish): Eye-healthy diet patterns support retinal metabolism; see supplements below. Purpose: supportive care. Mechanism: antioxidants and omega-3s.

17. Driving and hazard counseling: Discuss safe driving thresholds during active bleeding or edema. Purpose: safety. Mechanism: risk reduction. PMC

18. Family genetic counseling/testing: Finds at-risk relatives early so monitoring can start before vision loss. Purpose: prevention/early detection. Mechanism: identify CAPN5 carriers. Ophthalmology Advisor

19. Infection precautions on immunosuppressants: Hand hygiene, food safety, sick-contact avoidance. Purpose: reduce severe infections. Mechanism: lowers pathogen exposure when on biologics. FDA Access Data

20. Sunset-scheduling for delayed-release prednisone (if used): Aligns steroid timing to symptom peaks when appropriate. Purpose: symptom control with less dose. Mechanism: chronotherapy. FDA Access Data

---

Drug treatments

Important: The drugs below are not “approved for CAPN5 disease” specifically (there are no disease-specific approvals). They are used to treat features of ADNIV—uveitis, macular edema, and neovascularization—based on FDA-approved indications for those features or on accepted off-label practice. Always treat infection first if suspected and follow retina/uveitis specialist guidance.

1. Adalimumab (Humira/Hulio biosimilar)—subcutaneous anti-TNF used for non-infectious intermediate, posterior, and panuveitis in adults and children ≥2y. Class: biologic TNF-α inhibitor. Typical dose (adult): 80 mg once, then 40 mg every other week. Purpose: quiet chronic uveitis. Mechanism: blocks TNF-α to reduce inflammation. Key risks: serious infection, TB reactivation. FDA Access Data+2FDA Access Data+2

2. Dexamethasone 0.7 mg intravitreal implant (Ozurdex)—for posterior non-infectious uveitis and some edemas. Class: corticosteroid implant. Dosed in-office as needed. Purpose: reduce intraocular inflammation/edema. Mechanism: broad anti-inflammatory effect. Risks: IOP rise, cataract; contraindicated with ocular infection. FDA Access Data+1

3. Fluocinolone acetonide 0.59 mg implant (Retisert)—for chronic NIU-posterior; surgical implant releases steroid for ~30 months. Class: corticosteroid implant. Purpose: long-term control. Mechanism: sustained steroid delivery. Risks: cataract, IOP rise, need for glaucoma surgery. FDA Access Data+1

4. Fluocinolone acetonide 0.18/0.19 mg implant (Yutiq / Iluvien)—for chronic NIU-PS (Yutiq) and DME/NIU-PS (Iluvien label now includes NIU-PS). Class: corticosteroid implant. Purpose: long-duration control with smaller device. Risks: IOP rise, cataract. FDA Access Data+2FDA Access Data+2

5. Triamcinolone acetonide (Triesence) intravitreal—for uveitis and for vitrectomy visualization. Class: corticosteroid suspension. Typical: 4 mg intravitreal for uveitis. Purpose: short-to-medium term inflammation control. Risks: IOP rise, cataract, endophthalmitis risk with injections. FDA Access Data+1

6. Prednisone/Prednisolone (systemic)—short courses for active flares when vision-threatening. Class: systemic corticosteroid. Adult doses vary (e.g., 0.5–1 mg/kg/day tapered). Purpose: rapid inflammation control. Risks: infection, glucose, bone, ocular hypertension/cataract (monitor). FDA Access Data+1

7. Methotrexate (systemic)—steroid-sparing immunomodulator for chronic non-infectious uveitis. Class: antimetabolite/DMARD. Typical weekly dosing (e.g., 7.5–25 mg) with folic acid. Purpose: reduce steroid need and prevent flares. Risks: liver, marrow, teratogenic—monitor labs. FDA Access Data+1

8. Mycophenolate mofetil (CellCept)—steroid-sparing agent used widely in uveitis. Class: antimetabolite. Typical: 1–1.5 g twice daily. Purpose: long-term control. Risks: infection, GI, teratogenicity. FDA Access Data+1

9. Cyclosporine (Sandimmune/Neoral)—calcineurin inhibitor for refractory uveitis. Class: calcineurin inhibitor. Dosing varies; monitor levels/renal function. Purpose: T-cell suppression. Risks: nephrotoxicity, hypertension; drug interactions (grapefruit). FDA Access Data+1

10. Tacrolimus (Prograf)—alternative calcineurin inhibitor when cyclosporine not tolerated. Class: calcineurin inhibitor. Purpose: T-cell suppression. Risks: nephrotoxicity, neurotoxicity; strong CYP3A interactions. FDA Access Data+1

11. Sirolimus (Rapamune)—mTOR inhibitor sometimes used systemically for uveitis when other agents fail. Class: mTOR inhibitor immunosuppressant. Purpose: steroid-sparing. Risks: hyperlipidemia, mucositis, infection. (Label for transplant; off-label in uveitis.) FDA Access Data

12. Infliximab (Remicade)—IV anti-TNF for severe refractory non-infectious uveitis in practice. Class: monoclonal anti-TNF. Purpose: control scleritis/uveitis when adalimumab insufficient. Risks: serious infection, TB. FDA Access Data+1

13. Tocilizumab (Actemra)—IV/SC anti-IL-6R used off-label for uveitic macular edema resistant to TNF inhibitors. Class: biologic anti-IL-6 receptor. Risks: infections, lab abnormalities; TB/HBV screening. FDA Access Data+1

14. Rituximab (Rituxan)—anti-CD20 B-cell therapy used rarely for refractory autoimmune eye disease. Class: monoclonal antibody. Risks: infusion reactions, PML (rare). FDA Access Data+1

15. Ranibizumab (Lucentis) intravitreal—for retinal neovascular disorders and diabetic macular edema; used off-label to suppress pathologic neovascularization or edema in ADNIV when present. Class: anti-VEGF. Risks: endophthalmitis; rare inflammation. FDA Access Data+1

16. Aflibercept (Eylea) intravitreal—anti-VEGF trap for neovascular disease and DME; similar rationale to ranibizumab. Class: anti-VEGF fusion protein. Risks: as above. FDA Access Data+1

17. Brolucizumab (Beovu) intravitreal—potent anti-VEGF; has label warnings for retinal vasculitis/occlusion—use with caution in inflammatory eyes. Class: anti-VEGF. FDA Access Data+1

18. Faricimab (Vabysmo) intravitreal—dual Ang-2/VEGF-A inhibitor for AMD/DME; sometimes considered when edema is prominent; inflammation risk profile considered. Class: bispecific mAb. (Label source.) FDA Access Data

19. Acetazolamide (oral)—adjunct for cystoid macular edema in some retinopathies. Class: carbonic anhydrase inhibitor. Typical 250–500 mg 2–3×/day (renal dosing). Risks: paresthesias, kidney stones. FDA Access Data

20. Dorzolamide (topical)—topical CAI sometimes used for CME adjunct or IOP control. Class: carbonic anhydrase inhibitor eye drops. Risks: local irritation; caution in sulfonamide allergy. FDA Access Data

---

Dietary molecular supplements

1. AREDS2 formula (lutein+zeaxanthin, zinc, copper; no beta-carotene): Eye-specific antioxidant mix that supports macular health; often chosen over beta-carotene in smokers. Typical: per AREDS2 label daily. Function: antioxidant/anti-inflammatory support. Mechanism: quenches oxidative stress.

2. Omega-3 fatty acids (EPA/DHA): Support retinal cell membranes and may modulate inflammation; 1–2 g/day commonly used in eye health. Function: anti-inflammatory lipid mediators. Mechanism: resolvins/protectins pathways. (General ocular nutrition evidence.)

3. Zeaxanthin alone (macular pigment): Enhances macular pigment density, improving glare/contrast. Dose: often 2 mg–20 mg/day in supplements. Function: blue-light filtration. Mechanism: pigment deposition in macula.

4. Lutein: As above; typically 10 mg/day in AREDS-style supplements. Function/mechanism: antioxidant/macronutrient for macula.

5. Vitamin D (replete deficiency): Low vitamin D is linked to some autoimmune activity; clinicians often correct deficiency. Dose: per lab-guided repletion. Function: immune modulation. Mechanism: vitamin D receptor signaling. (General evidence.) PMC

6. Curcumin: Experimental anti-inflammatory; small studies suggest uveitis symptom reduction; bioavailability matters. Typical 500–1000 mg/day curcuminoids with enhanced formulations. Mechanism: NF-κB/COX modulation.

7. Resveratrol: Antioxidant with retinal protective signals in lab/animal models; human ocular data limited. Dose: 100–500 mg/day in supplements. Mechanism: SIRT/antioxidant pathways.

8. N-Acetylcysteine (NAC): Glutathione precursor; studied for retinal oxidative stress. Common 600–1200 mg/day. Mechanism: boosts antioxidant defenses.

9. Zinc (with copper): Part of AREDS2; avoid excess zinc without copper. Dose: per AREDS2 (e.g., 25–80 mg zinc with 2 mg copper depending on product). Mechanism: antioxidant enzyme cofactor.

10. Bilberry (anthocyanins): Traditional vision support; scientific evidence mixed; use as adjunct only. Typical 80–160 mg anthocyanins/day. Mechanism: flavonoid antioxidant effects.

Always confirm supplement safety with your clinician, especially with methotrexate, cyclosporine, tacrolimus, or biologics (interaction risks).

---

Immunity booster / regenerative / stem-cell” drugs

There are no FDA-approved stem cell drugs for retinal regeneration in ADNIV, and the FDA has warned about severe injuries (including blindness) from unapproved stem-cell injections into the eye. Accordingly, the safest, evidence-based approach is to use approved immunomodulators (already listed) and avoid unproven “regenerative” offerings. EyeWiki+1

For completeness, six FDA-approved systemic immunomodulators (used to modulate immunity—not “boost” it) that retina/uveitis specialists may select in refractory cases (off-label for ADNIV but evidence-based for non-infectious uveitis): adalimumab, infliximab, tocilizumab, methotrexate, mycophenolate, cyclosporine/tacrolimus. Dosing/risks appear above and on their FDA labels. These agents suppress pathologic immune activity to protect vision. FDA Access Data+5FDA Access Data+5FDA Access Data+5

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

---

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

---

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

---

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

---

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.