Central Areolar Choroidal Sclerosis

Central areolar choroidal sclerosis is an older name that doctors used for a rare inherited (genetic) macular disease now most often called central areolar choroidal dystrophy (CACD). It mainly damages the macula (the center part of the retina that you use for reading and recognizing faces). Over years, the disease can cause a round, well-defined “bare” area in the center of the macula because the retinal pigment epithelium (RPE), the choriocapillaris (tiny blood layer under the retina), and the light-sensing cells gradually waste away (atrophy). This usually leads to slowly progressive central vision loss, while side (peripheral) vision is often much better. NCBI+2EyeWiki+2

Central areolar choroidal sclerosis is an older name that many people use for central areolar choroidal dystrophy (CACD). It is a rare, inherited macular disease. “Central” means the middle of the retina (the macula, used for sharp reading vision). “Areolar” means a clear, round area. “Choroidal” means the blood-rich layer under the retina. “Sclerosis” in this name points to long-term damage and thinning. In CACD, the cells in the macula slowly wear out, including the retinal pigment epithelium (RPE) and nearby support tissues, causing progressive loss of central vision over years. There is no proven cure yet, so care focuses on protecting the eyes, monitoring changes, treating complications, and using low-vision help. EyeWiki+1

Another names

These names have been used in books and papers for the same or very similar condition: central areolar choroidal dystrophy (CACD), central areolar choroidal atrophy, familial central areolar choroidal atrophy, central senile areolar choroidal dystrophy/atrophy, and choroidal angio-sclerosis. Many sources note that “central areolar choroidal sclerosis” is an older label and “CACD” is preferred today. EyeWiki+2JAMA Network+2

Types

• Stage 1 (very early CACD): small, subtle RPE color/pigment changes near the fovea (center of the macula). EyeWiki

• Stage 2 (early atrophy starts): a round/oval pale area appears, but the edges are still not sharply clear. EyeWiki

• Stage 3 (clear patches of atrophy): one or more well-outlined patches of RPE loss develop (often around, not exactly on, the fovea at first). EyeWiki

• Stage 4 (advanced CACD): the central atrophic area becomes well-defined and reaches the fovea, causing strong loss of central vision. EyeWiki

Causes

Important note (simple): “Central areolar choroidal sclerosis” is often used to mean CACD (a genetic dystrophy), but doctors must also rule out other diseases that can look similar. So, below are (A) proven genetic causes linked to CACD and (B) common look-alike causes listed in clinical references. EyeWiki+1

1. PRPH2 gene mutation (overall): the most classic cause of autosomal-dominant CACD in many families; PRPH2 affects photoreceptor outer-segment structure, and damaging variants can lead to progressive macular atrophy. EyeWiki+2PubMed+2

2. PRPH2 variant p.Arg142Trp: a well-known PRPH2 change reported in autosomal-dominant CACD families. EyeWiki+1

3. PRPH2 variant p.Arg172Trp: another reported PRPH2 cause; some sources note it may be linked with broader cone/cone-rod involvement in certain cases. EyeWiki+1

4. PRPH2 variant p.Arg172Gln: reported among PRPH2 variants associated with CACD. EyeWiki

5. PRPH2 variant p.Arg195Leu: reported in human CACD and used in research models to study disease mechanisms. EyeWiki+1

6. PRPH2 variant p.Leu307fsX83 (frameshift): a truncating type of PRPH2 change listed among CACD-associated variants. EyeWiki

7. GUCY2D gene mutation (CACD2 in some classifications): some families have CACD due to GUCY2D variants (a different genetic pathway than PRPH2). Genome Browser+1

8. GUCA1A gene mutation: reported among genes implicated in inherited CACD in genetic reviews. PubMed

9. CDHR1 gene mutation: reported among genes implicated in inherited CACD in genetic reviews. PubMed

10. ABCA4 gene mutation: reported among genes discussed in CACD genetic networks (and also known for Stargardt disease, which can mimic CACD). PubMed

11. TTLL5 gene mutation: reported among genes implicated in inherited CACD in genetic reviews. PubMed

12. Age-related macular degeneration (AMD) with geographic atrophy: a major look-alike; advanced atrophy in AMD can resemble late-onset CACD, and imaging plus family history/genetics help separate them. EyeWiki+1

13. Central areolar dystrophy of the RPE: another listed differential cause of a central atrophic macular lesion. EyeWiki

14. Myopic degeneration (pathologic myopia changes): high myopia can cause degenerative macular changes that may look like central atrophy. EyeWiki

15. Stargardt disease: an inherited macular dystrophy that can be confused with CACD, especially when atrophy becomes advanced. EyeWiki+1

16. Cone dystrophy: cone-predominant retinal dystrophy can cause central vision loss and macular changes that overlap with CACD patterns. EyeWiki

17. North Carolina macular dystrophy: a hereditary macular disorder included in differential diagnosis lists for CACD-like appearances. EyeWiki

18. Best disease (Best vitelliform macular dystrophy): can mimic CACD in some stages or in late atrophic phases, so it is listed as a differential diagnosis. EyeWiki+1

19. Pattern dystrophy: PRPH2 can also cause pattern dystrophy; pattern dystrophy is a known CACD look-alike and part of the differential list. EyeWiki+1

20. Chloroquine / hydroxychloroquine retinopathy (bull’s-eye maculopathy): medication toxicity can create a bull’s-eye/atrophic macular pattern and must be ruled out with history and tests. EyeWiki

Symptoms

1. Slowly worsening central vision: you may notice the center of what you look at is not as sharp as before, and it tends to worsen over years as macular atrophy grows. Orpha.net+1

2. Blurred vision for reading: reading small text becomes hard because the macula is needed for fine detail. Retina UK

3. Difficulty recognizing faces: face recognition uses central vision; when central vision drops, faces can look unclear. Retina UK

4. Central scotoma (a missing spot in the center): people can develop a “blank” or “missing” patch in the middle of vision that matches the atrophic area. EyeWiki+1

5. Distorted vision (straight lines look bent): when macular cells are stressed or unevenly damaged, lines may look wavy. Retina UK

6. Need brighter light to read: damaged macula often needs stronger lighting for the same task. Retina UK

7. Reduced color sense: color vision may drop because cones in the macula are affected. EyeWiki+1

8. Trouble with fine detail work: tasks like threading a needle or reading on a phone become difficult. Orpha.net+1

9. Poor contrast sensitivity: even if letters are big, low-contrast text (gray on white) may be hard to see. Retina UK

10. Vision problems in both eyes: CACD is typically bilateral and fairly symmetric, so both eyes can be affected over time (often not perfectly equally). EyeWiki+1

11. Symptoms may start in young to middle adulthood: many sources describe onset often from the 20s–40s (but it can vary, including later onset that resembles AMD). EyeWiki+2Retina UK+2

12. “Patchy” central vision early on: in earlier stages, you may have mild blur or small disturbed spots before a large central scotoma appears. EyeWiki+1

13. Difficulty driving (especially reading signs): reading road signs depends on central detail vision, which may decline as the fovea becomes involved. Retina UK

14. Relatively preserved side vision: many people keep peripheral vision better than central vision, because CACD mainly targets the macula. Retina UK+1

15. Low-vision impact in later stages: when the atrophy reaches the fovea (advanced stage), central vision loss can become severe and daily tasks may need low-vision support. EyeWiki+1

Diagnostic tests

Physical exam (done by an eye doctor)

1. Best-corrected visual acuity test: checks how sharp your central vision is with the best glasses/lens correction; CACD typically lowers central acuity over time. Orpha.net+1

2. Refraction (glasses check): helps prove the blur is not only from needing glasses; in CACD, vision stays reduced even with correct lenses as disease progresses. Retina UK

3. Dilated fundus examination: the doctor looks at the macula through a dilated pupil to see pigment changes or central atrophy typical of CACD stages. EyeWiki+1

4. Color vision testing (clinic test): simple color plate tests can detect reduced color discrimination that can happen with macular cone damage. EyeWiki+1

Manual / functional vision tests (simple, patient-based tests)

5. Amsler grid test: a small grid you look at to detect distortion or missing central spots, which fits central macular disease patterns. Retina UK

6. Central visual field test (standard perimetry): measures a central scotoma and how it grows with disease progression. EyeWiki

7. Microperimetry: maps light sensitivity specifically across the macula to show functional loss even near borders of atrophy (useful in macular dystrophies). IOVS

8. Contrast sensitivity test: checks how well you see faint differences; central macular disease often reduces contrast sensitivity. Retina UK

Lab and pathological tests (to confirm cause and rule out look-alikes)

9. Genetic testing for PRPH2: if imaging and family history suggest CACD, genetic analysis can confirm a PRPH2 mutation and support the diagnosis. EyeWiki+2PubMed+2

10. Inherited retinal disease gene panel (multi-gene test): because CACD can be genetically heterogeneous, panels may include PRPH2 and other implicated genes discussed in research reviews. PubMed+1

11. Targeted testing for GUCY2D (when suspected): some CACD cases are linked to GUCY2D; testing helps confirm that subtype. Genome Browser+1

12. Family screening / pedigree review (clinical genetics workup): documenting affected relatives supports an inherited pattern and helps separate CACD from non-genetic causes like AMD. EyeWiki+1

Electrodiagnostic tests (measure retina function with electrodes)

13. Full-field ERG (electroretinogram): measures summed retinal electrical responses; it can be normal in very localized macular disease, but abnormalities (especially cone pathway changes) have been reported in CACD. EyeWiki+1

14. Multifocal ERG (mfERG): measures many small macular areas separately, helping detect macular dysfunction even outside obvious atrophy. EyeWiki+1

15. Electro-oculogram (EOG): sometimes used to evaluate RPE function; reports note it may be normal in CACD, which can help in differential diagnosis. Radboud Repository+1

Imaging tests (pictures/scans of retina and choroid)

16. Fundus photography: documents the appearance and size of macular changes over time for follow-up. PubMed+1

17. Fundus autofluorescence (FAF): shows RPE stress/loss patterns; studies describe FAF as helpful for defining disease extent and monitoring progression in choroidal sclerosis/CACD. PMC+2EyeWiki+2

18. Optical coherence tomography (OCT): a cross-section scan that shows loss of outer retinal layers and RPE/Bruch’s changes that support CACD staging and help separate from AMD. EyeWiki+1

19. Fluorescein angiography (FA): dye test that can outline atrophic areas and choroidal vessel visibility, especially in later stages. EyeWiki+1

20. Indocyanine green angiography (ICGA): dye imaging focused more on choroidal circulation; clinical references describe ICGA patterns in CACD. EyeWiki+1

Non-pharmacological treatments (therapies + others)

1) Regular retina specialist follow-up (planned eye visits). Purpose: catch new problems early (like sudden worsening or new bleeding). Mechanism: exams and scans (often OCT) track how the macula is changing, so the plan can be adjusted quickly. EyeWiki+1

2) Low-vision rehabilitation (vision training). Purpose: help you keep independence in school/work/home. Mechanism: a vision rehab team teaches safer reading methods, daily-task tricks, and how to use remaining side vision better. American Academy of Ophthalmology+1

3) Optical magnifiers (handheld/stand magnifiers). Purpose: make print and objects look larger. Mechanism: lenses enlarge images so the healthier retina can “catch” more detail. American Academy of Ophthalmology+1

4) Electronic magnification (CCTV/video magnifiers). Purpose: reading and near work with less strain. Mechanism: a camera enlarges text onto a screen, and contrast can be changed to make letters easier to see. American Academy of Ophthalmology+1

5) Large-print and high-contrast settings. Purpose: easier reading with less fatigue. Mechanism: bigger fonts and bold contrast reduce how much fine central detail you need to recognize words. American Academy of Ophthalmology+1

6) Better lighting (bright, even, non-glare light). Purpose: improve clarity and reduce “wash-out.” Mechanism: strong, even light increases contrast and reduces shadows that make low vision worse. American Academy of Ophthalmology+1

7) Glare control (hats, visors, anti-glare filters). Purpose: reduce bright-light discomfort and improve contrast. Mechanism: cutting glare stops scattered light from reducing the sharpness of what you see. American Academy of Ophthalmology+1

8) UV-blocking sunglasses outdoors. Purpose: protect eyes from ultraviolet light and reduce glare. Mechanism: UV filters reduce light stress and discomfort (this does not “cure” CACD, but it can make vision more comfortable). EyeWiki+1

9) Amsler grid or home distortion checks. Purpose: notice new distortion early. Mechanism: a simple grid helps you detect new wavy lines or missing spots that can signal a complication needing urgent care. EyeWiki+1

10) Reading strategies (line guides, finger tracking). Purpose: keep place on the page. Mechanism: guides reduce skipping lines when central vision is weak. American Academy of Ophthalmology+1

11) Assistive technology (screen readers, text-to-speech). Purpose: access books and digital content. Mechanism: the device reads text aloud so vision is not the only way to learn. American Academy of Ophthalmology+1

12) Driving safety plan. Purpose: prevent accidents. Mechanism: regular vision checks + adapting (daytime driving only, avoiding glare/night driving) and stopping driving when unsafe. American Academy of Ophthalmology+1

13) Fall-prevention at home. Purpose: reduce injuries when vision is reduced. Mechanism: bright lights, clear walkways, contrast tape on stairs improve navigation. American Academy of Ophthalmology+1

14) School/work accommodations. Purpose: protect learning and productivity. Mechanism: seating close to board, digital notes, extra time, and magnification tools reduce the “vision load.” American Academy of Ophthalmology+1

15) Genetic counseling (family planning + expectations). Purpose: understand inheritance risk and what to monitor. Mechanism: counselors explain family patterns and guide testing decisions. Genetic Diseases Info Center+1

16) Emotional support (counseling/support groups). Purpose: reduce stress and isolation. Mechanism: coping skills and peer support help people adjust to long-term vision change. American Academy of Ophthalmology+1

17) Healthy sleep routine. Purpose: support overall brain and eye comfort. Mechanism: good sleep reduces fatigue that can worsen how hard it feels to see and focus. American Academy of Ophthalmology+1

18) Physical activity (regular walking/exercise). Purpose: support blood vessel health. Mechanism: better heart and vessel health supports overall eye health (not a cure, but helpful for general risk control). Genetic Diseases Info Center+1

19) Control systemic risks (blood pressure, diabetes, smoking). Purpose: reduce extra damage risks to retinal blood supply. Mechanism: healthier vessels and less inflammation reduce added stress on eye tissues. Genetic Diseases Info Center+1

20) Emergency plan (know danger signs). Purpose: avoid delayed treatment if a complication happens. Mechanism: knowing red-flags (sudden distortion, big new blind spot, flashes/floaters, severe pain/redness) leads to faster care. EyeWiki+1

Drug treatments

Important note: No FDA-approved drug “cures” CACD itself. The medicines below are used to treat complications (like choroidal neovascularization/wet-type changes), slow similar atrophy in other diseases (GA/AMD), or support comfort—only an ophthalmologist can decide what fits your case. EyeWiki+1

1) Ranibizumab (LUCENTIS). Class: anti-VEGF biologic. Dose/time (label example): 0.5 mg (0.05 mL) intravitreal monthly. Purpose: treat abnormal leaking blood vessels (CNV). Mechanism: blocks VEGF signals that drive new leaky vessels. Side effects (important): infection inside eye, retinal detachment, eye pressure rise. FDA Access Data

2) Aflibercept (EYLEA). Class: anti-VEGF. Dose/time (label example): 2 mg (0.05 mL) every 4 weeks for initial doses, then often every 8 weeks. Purpose/mechanism: reduces leakage and bleeding from abnormal vessels by blocking VEGF activity. Side effects: endophthalmitis risk, retinal tear/detachment risk, eye pressure rise. FDA Access Data

3) Aflibercept 8 mg (EYLEA HD). Class: anti-VEGF. Dose/time (label example): 8 mg (0.07 mL) monthly for first 3 doses, then every 8–16 weeks in some indications. Purpose: longer-interval control of leaking vessels/swelling (doctor decides). Mechanism: stronger dose VEGF binding. Side effects: similar injection-related serious eye risks. FDA Access Data

4) Faricimab (VABYSMO). Class: anti-VEGF/anti-Ang-2 biologic. Dose/time (label example): 6 mg (0.05 mL) monthly for first 4 doses, then intervals based on disease activity. Purpose: treat wet-type vessel leakage. Mechanism: blocks VEGF-A and Ang-2 pathways to reduce leakage and inflammation signals. Side effects: infection risk, inflammation, pressure rise. FDA Access Data

5) Brolucizumab (BEOVU). Class: anti-VEGF. Dose/time (label example): 6 mg (0.05 mL) with loading then every 8–12 weeks (label schedules vary by version). Purpose: control wet-type CNV leakage. Mechanism: blocks VEGF-A. Side effects: inflammation/vasculitis risks have been reported; needs careful specialist monitoring. FDA Access Data+1

6) Ranibizumab implant system (SUSVIMO). Class: ocular drug-delivery implant (ranibizumab). Time (label concept): refill-exchange planned about every 24 weeks, and missed refills are handled with a new schedule. Purpose: longer-term delivery with fewer injections in selected patients. Mechanism: continuous release of ranibizumab into the eye. Side effects: surgical-site complications plus injection-type risks. FDA Access Data

7) Ranibizumab-nuna (BYOOVIZ, biosimilar). Class: anti-VEGF biosimilar to ranibizumab. Dose/time (label example): 0.5 mg (0.05 mL) monthly (indication-dependent). Purpose/mechanism: similar to ranibizumab—blocks VEGF to reduce leaking vessels. Side effects: similar intravitreal injection risks. FDA Access Data

8) Ranibizumab-eqrn (CIMERLI, biosimilar). Class: anti-VEGF biosimilar. Dose/time (label example): 0.5 mg (0.05 mL) monthly (indication-dependent). Purpose: treat wet-type vessel leakage when present. Mechanism: VEGF inhibition. Side effects: injection-related serious eye risks. FDA Access Data

9) Pegcetacoplan (SYFOVRE). Class: complement inhibitor. Dose/time (label): 15 mg (0.1 mL) intravitreal every 25–60 days for geographic atrophy (GA) secondary to AMD. Purpose: slows GA growth in AMD (not proven for CACD, but sometimes discussed when atrophy is the main issue). Mechanism: reduces complement-driven inflammation. Side effects: endophthalmitis risk, retinal detachment risk, inflammation, and wet-AMD conversion risk. FDA Access Data

10) Avacincaptad pegol (IZERVAY). Class: complement inhibitor. Dose/time (FDA review/label summary): 2 mg (0.1 mL of 20 mg/mL) intravitreal monthly (about every 28 ± 7 days) for GA secondary to AMD. Purpose: slow GA progression in AMD (not proven for CACD). Mechanism: blocks complement pathway activity. Side effects: injection risks and inflammation risks. FDA Access Data

11) Verteporfin (VISUDYNE) for photodynamic therapy (PDT). Class: photosensitizing drug used with a laser procedure. Dose/time (label): given by IV infusion and then activated with eye laser in specific protocols. Purpose: close abnormal leaking vessels in some CNV cases. Mechanism: light activation creates local vessel closure effects. Side effects: light sensitivity reactions and vision changes; needs strict precautions. FDA Access Data

12) Bevacizumab (AVASTIN) (off-label in the eye). Class: anti-VEGF monoclonal antibody (cancer drug). Dose/time: ocular use is off-label; retina specialists sometimes use it intravitreally for CNV. Purpose/mechanism: VEGF blockade to reduce leakage/bleeding. Side effects: injection-related eye risks; dosing is not the cancer label dosing. FDA Access Data

13) Dexamethasone intravitreal implant (OZURDEX). Class: corticosteroid implant. Dose/time (label concept): one implant into the affected eye for approved edema/inflammation indications (repeat timing decided by specialist). Purpose: reduce retinal swelling or inflammation if present. Mechanism: lowers inflammatory signals and leakage. Side effects: cataract, eye pressure rise, infection risk. FDA Access Data

14) Fluocinolone acetonide intravitreal implant (ILUVIEN). Class: long-acting corticosteroid implant. Dose/time: implant placed into the eye for certain chronic retinal edema conditions. Purpose: long-term inflammation control when appropriate. Mechanism: steroid anti-inflammatory effect over time. Side effects: high eye pressure/glaucoma risk and cataract risk. FDA Access Data

15) Prednisolone acetate eye drops (PRED FORTE). Class: topical corticosteroid. Dose/time (label example): dosing can be frequent at first, then tapered by doctor. Purpose: reduce eye inflammation (for example after procedures or with surface inflammation). Mechanism: lowers inflammatory chemicals. Side effects: eye pressure rise, infection risk with misuse. FDA Access Data

16) Ketorolac ophthalmic (ACULAR / ketorolac drops). Class: NSAID eye drop. Dose/time: depends on product/indication (often multiple times daily). Purpose: reduce pain and inflammation on the eye surface (for example around surgery). Mechanism: blocks prostaglandin formation. Side effects: stinging, delayed healing in some cases. DailyMed+1

17) Moxifloxacin ophthalmic (VIGAMOX). Class: antibiotic eye drop. Dose/time: depends on infection/doctor plan. Purpose: prevent or treat bacterial infection risk (sometimes around procedures). Mechanism: kills bacteria by blocking DNA replication enzymes. Side effects: irritation, allergy in some people. FDA Access Data

18) Polymyxin B/Trimethoprim (POLYTRIM). Class: antibiotic combo eye drop. Purpose: treats common bacterial eye infections if they occur. Mechanism: attacks bacteria in two different ways. Side effects: irritation and allergy. FDA Access Data

19) Cyclosporine ophthalmic emulsion (RESTASIS). Class: topical immunomodulator. Dose/time (label): one drop twice daily in each eye about 12 hours apart. Purpose: improve dry eye when inflammation suppresses tear production (comfort and clearer vision). Mechanism: reduces T-cell driven inflammation on the eye surface. Side effects: burning/stinging. FDA Access Data

20) Lifitegrast ophthalmic (XIIDRA). Class: anti-inflammatory eye drop for dry eye disease. Dose/time (label): one drop twice daily about 12 hours apart. Purpose: reduce dry-eye discomfort and blurred vision from surface dryness. Mechanism: blocks an immune adhesion pathway (LFA-1/ICAM-1) that drives inflammation. Side effects: irritation and unusual taste. FDA Access Data

Dietary molecular supplements (supportive; not a cure)

Important note: Supplements cannot cure CACD. Some people use AREDS/AREDS2-type nutrients because they are proven in AMD, not CACD. Always ask your eye doctor first, especially if you take blood thinners or have kidney disease. National Eye Institute+1

1) Vitamin C. Typical AREDS amount: 500 mg/day. Function: antioxidant support. Mechanism: helps neutralize oxidative stress that can damage tissues. Too much may upset stomach in some people. National Eye Institute

2) Vitamin E. Typical AREDS amount: 400 IU/day (AREDS used 400 IU; safety questions exist for some people). Function: antioxidant support. Mechanism: helps protect cell membranes from oxidative damage. Discuss safety with a clinician if you have medical conditions. National Eye Institute+1

3) Zinc. Typical AREDS amount: 80 mg/day (with copper). Function: supports retinal metabolism and antioxidant enzymes. Mechanism: helps enzymes that protect cells from oxidative injury. High zinc without copper can cause copper deficiency. National Eye Institute

4) Copper. Typical AREDS amount: 2 mg/day. Function: prevents copper deficiency that can happen when zinc is high. Mechanism: supports normal blood and nerve function while balancing zinc use. National Eye Institute

5) Lutein. Typical AREDS2 amount: 10 mg/day (common AREDS2). Function: macular pigment support. Mechanism: helps filter damaging light and supports antioxidant activity in the macula. National Eye Institute

6) Zeaxanthin. Typical AREDS2 amount: 2 mg/day. Function: works with lutein in macular pigment. Mechanism: supports light filtering and antioxidant effects in retinal tissues. National Eye Institute

7) Omega-3 fatty acids (DHA/EPA). Function: supports cell membranes and may support overall eye health. Mechanism: anti-inflammatory lipid signaling and membrane support; evidence is stronger for diet patterns than for “curing” macular disease. National Eye Institute

8) Vitamin D. Function: general immune and bone support; some people use it for overall health. Mechanism: hormone-like vitamin that affects inflammation pathways; not proven to treat CACD directly. Genetic Diseases Info Center+1

9) Vitamin B12 + folate (when deficient). Function: nerve and blood support. Mechanism: supports normal nerve function and red blood cell production; only useful if you truly have low levels. Genetic Diseases Info Center+1

10) N-acetylcysteine (NAC). Function: antioxidant “glutathione support.” Mechanism: helps the body rebuild glutathione, a key antioxidant; evidence for inherited macular dystrophy is limited, so use only with clinician guidance. EyeWiki+1

Drugs often mentioned for “immunity / regenerative / stem-cell” ideas

Key truth: There is no FDA-approved stem-cell drug that restores the macula in CACD today. “Regenerative” treatments are mostly research/clinical trials right now, but a few approved medicines show how regenerative or immune pathways can be used in eye care. EyeWiki+1

1) Cenegermin (OXERVATE) — regenerative example (cornea, not retina). Class: nerve growth factor (NGF) eye drop for neurotrophic keratitis. Dose/time (label): 1 drop, 6 times/day for 8 weeks. Purpose: helps corneal healing when nerve supply is damaged. Mechanism: supports nerve and surface healing pathways. Side effects: eye pain/irritation. FDA Access Data

2) Pegcetacoplan (SYFOVRE) — immune/complement pathway example. Class: complement inhibitor. Dose/time (label): 15 mg intravitreal every 25–60 days for GA in AMD. Purpose: slows atrophy growth in AMD (not proven for CACD). Mechanism: reduces complement-driven inflammation. Side effects: serious injection risks, inflammation. FDA Access Data

3) Avacincaptad pegol (IZERVAY) — immune/complement pathway example. Class: complement inhibitor. Dose/time: 2 mg monthly for GA in AMD (FDA review/label summary). Purpose: slows GA progression in AMD (not proven for CACD). Mechanism: complement pathway control. Side effects: injection risks, inflammation. FDA Access Data

4) Cyclosporine ophthalmic (RESTASIS) — immune control on eye surface. Class: topical immunomodulator. Dose/time (label): 1 drop twice daily. Purpose: improves tear production when inflammation causes dry eye. Mechanism: reduces T-cell inflammation. Side effects: burning. FDA Access Data

5) Lifitegrast ophthalmic (XIIDRA) — immune control on eye surface. Class: anti-inflammatory dry-eye medicine. Dose/time (label): 1 drop twice daily. Purpose: reduces symptoms and signs of dry eye. Mechanism: blocks immune cell binding signals. Side effects: irritation, strange taste. FDA Access Data

6) Voretigene neparvovec (LUXTURNA) — gene therapy example (specific gene disease, not CACD). Class: retinal gene therapy for confirmed RPE65-related retinal dystrophy. Purpose: improves vision in that specific genetic condition (not used for CACD unless the gene matches). Mechanism: delivers a working gene copy to retinal cells. Side effects: surgery-related risks and eye inflammation risks. U.S. Food and Drug Administration

Surgeries/procedures (what they are, and why done)

1) Intravitreal injection procedure. Why: if CACD develops a wet-type complication (CNV), injections may protect vision. What happens: medicine is placed inside the eye with sterile technique. FDA Access Data+1

2) Implant surgery (SUSVIMO). Why: for selected patients needing long-term anti-VEGF delivery. What happens: a small implant is placed surgically and later refilled by a special procedure. FDA Access Data

3) Photodynamic therapy (PDT) with verteporfin. Why: sometimes used to close abnormal vessels in certain CNV patterns. What happens: IV verteporfin is given, then a laser activates it in the eye area. FDA Access Data

4) Cataract surgery (if cataract develops). Why: cataract can further blur vision on top of CACD. What happens: cloudy lens is removed and replaced with a clear artificial lens. (This does not fix macular damage.) EyeWiki+1

5) Vitrectomy (only if another problem exists, like traction or macular hole). Why: some separate retina problems need surgery to prevent worse vision loss. What happens: gel inside the eye is removed to relieve pulling or repair retina issues. EyeWiki+1

Prevention tips

1) Don’t smoke and avoid second-hand smoke. It lowers extra inflammation and vessel damage risk that can make eye disease harder to manage. Genetic Diseases Info Center+1

2) Protect eyes from harsh sun and glare. Use UV-blocking sunglasses and a hat outdoors to reduce discomfort and glare problems. American Academy of Ophthalmology+1

3) Control blood pressure. Healthy blood pressure supports healthy vessels that feed the eye. Genetic Diseases Info Center+1

4) Control diabetes (if present). Stable blood sugar helps protect retinal blood vessels. Genetic Diseases Info Center+1

5) Keep regular eye checks even if symptoms feel stable. CACD changes slowly, but complications can appear faster. EyeWiki+1

6) Use low-vision tools early (don’t “wait until it’s worse”). Early training helps you adapt better. American Academy of Ophthalmology+1

7) Make your home vision-friendly. Bright lighting, contrast tape, and clear walkways prevent falls and make daily life easier. American Academy of Ophthalmology+1

8) Eat a retina-friendly diet pattern. Leafy greens, colorful vegetables, and fish support overall eye nutrition (not a cure, but supportive). National Eye Institute+1

9) Discuss family screening and genetics. It helps relatives know when to get checked and what signs to watch. Genetic Diseases Info Center+1

10) Know emergency warning signs (and act fast). Sudden distortion, a new big dark spot, flashes/floaters, or severe pain/redness need urgent care. EyeWiki+1

When to see doctors (urgent vs soon)

Go urgent / same day if you get sudden vision drop, new strong distortion, a new large blind spot, flashing lights, many new floaters, a “curtain” over vision, severe eye pain, or marked redness (especially after any injection/procedure). These can signal bleeding, retinal tear/detachment, or infection that needs fast treatment. EyeWiki+2FDA Access Data+2

See a doctor soon (within days to weeks) if reading becomes noticeably harder, faces become harder to recognize, glare suddenly worsens, or you need new tools for daily tasks—this is the right time to adjust low-vision devices, school/work supports, and monitoring plans. American Academy of Ophthalmology+1

What to eat and what to avoid

1) Eat leafy greens (spinach, kale). They are natural sources of lutein/zeaxanthin-type nutrients that support macular pigment. National Eye Institute

2) Eat colorful vegetables (orange/red/yellow). These provide antioxidants that support overall tissue health. National Eye Institute+1

3) Eat fatty fish 1–2 times/week (if available). Fish provides omega-3 fats that support cell membranes. National Eye Institute

4) Choose nuts and seeds (small amounts). They provide healthy fats and vitamin E sources in food. National Eye Institute

5) Prefer whole grains over refined grains. Better metabolic control supports blood vessel health. Genetic Diseases Info Center+1

6) Use healthy oils (like olive oil) instead of trans fats. Supports heart/vessel health, which supports eye health. Genetic Diseases Info Center+1

7) Avoid smoking and nicotine exposure. This is one of the strongest lifestyle risk reducers for many eye problems. Genetic Diseases Info Center+1

8) Limit ultra-processed foods and sugary drinks. Helps weight and blood sugar control, protecting vessels. Genetic Diseases Info Center+1

9) Avoid “mega-dose” supplements without medical advice. High doses (like vitamin E, zinc) can be unsafe for some people, so discuss first. National Eye Institute+1

10) If you use AREDS/AREDS2-type supplements, follow a clinician’s guidance. These formulas are evidence-based for AMD categories, not for CACD, so your doctor should decide if it fits you. National Eye Institute+1

 FAQs

1) Is central areolar choroidal sclerosis the same as CACD? In many texts, yes—people use it as an older name for central areolar choroidal dystrophy, a hereditary macular atrophy condition. EyeWiki+1

2) Is it contagious? No. It is an inherited retinal disease, not an infection. Genetic Diseases Info Center

3) Does it cause complete blindness? It mainly damages central vision. Many people keep some side vision, but reading and face recognition can become very hard. Genetic Diseases Info Center+1

4) At what age does it start? Many descriptions say symptoms often appear in adulthood, though timing can vary between families. Genetic Diseases Info Center+1

5) Is there a cure? There is no proven cure yet. Care focuses on monitoring, low-vision support, and treating complications. EyeWiki+1

6) Can glasses cure it? Glasses can correct focusing problems, but they cannot repair macular tissue loss. Low-vision devices often help more than standard glasses. American Academy of Ophthalmology+1

7) What is the macula and why does it matter? The macula is the center of the retina used for sharp detail (reading, faces). CACD mainly affects this area. Genetic Diseases Info Center+1

8) What scan is commonly used to follow it? OCT (optical coherence tomography) is commonly used to map retinal layers and track atrophy changes over time. EyeWiki

9) Why do doctors watch for “wet” changes? Sometimes abnormal vessels can grow and leak (CNV). This can cause sudden worsening but may respond to anti-VEGF treatment. FDA Access Data+1

10) Are eye injections safe? They are common and can be very helpful when indicated, but they carry rare serious risks like infection or retinal detachment, so they must be done by trained specialists with sterile technique. FDA Access Data+1

11) Do AREDS2 supplements treat CACD? AREDS2 nutrients are proven for certain AMD stages, not for CACD specifically. Some doctors may still discuss them case-by-case, especially when atrophy is present. National Eye Institute+1

12) What is the best “treatment” day-to-day? Low-vision rehabilitation plus assistive devices (magnifiers, electronic tools, high-contrast settings) often gives the biggest real-life benefit. American Academy of Ophthalmology+1

13) Can children get it? CACD is usually described as an adult-onset hereditary macular disorder, but inherited eye diseases can vary, so family history matters and an eye specialist should guide screening. Genetic Diseases Info Center+1

14) Should family members be checked? Often yes, especially if multiple relatives have similar vision loss. Genetic counseling and eye exams can help families plan and monitor early. Genetic Diseases Info Center+1

15) What research is happening now? Researchers study inherited retinal disease genetics and potential future treatments (including gene-based ideas). These are mostly not standard care for CACD today, but clinical trials may exist in some regions. EyeWiki+1

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: December 16, 2025.