Scleromalacia Perforans

Scleromalacia perforans is a rare eye problem where the tough white coat of the eye (the sclera) becomes very thin and weak in quiet, non-red areas. It happens without the usual signs of inflammation. In other words, the eye can look white and calm, yet the tissue is slowly melting. As the wall thins, the dark inner layer of the eye can show through, and the eye becomes prone to bulging and, in severe cases, to breaking with even minor injury. Doctors consider it a form of “necrotizing scleritis without inflammation.” It is most often linked to long-standing autoimmune disease, especially rheumatoid arthritis, and tends to be painless or only mildly irritating, which is why it can be missed until thinning is advanced. EyeWikiAAO-HNS+1

Scleromalacia perforans (SP) is a quiet, painless melting and thinning of the white coat of the eye (sclera) that can expose the dark uveal layer underneath and, if not treated promptly, can lead to perforation of the eye—most often in people with long-standing autoimmune disease such as rheumatoid arthritis. EyeWiki+1AAO

Scleromalacia perforans is a type of necrotizing scleritis without obvious surface inflammation. In everyday words, the white wall of the eye slowly melts and becomes very thin, yet the eye often looks white and calm and may not hurt, so the problem can be missed until a gray or dark area shows through. The dark patch is simply the normal uvea (the pigmented inner coat) becoming visible because the white wall over it has thinned. Over time, the thinning can bulge (staphyloma) or break (perforate), which is an emergency. SP is strongly linked to autoimmune diseases, especially rheumatoid arthritis (RA), and needs systemic (whole-body) evaluation and treatment, usually with immunosuppressive medicines and sometimes surgery to reinforce the wall. EyeWiki+1

How does it happen?

In many people, the body’s immune system mistakenly targets tiny blood vessels that feed the sclera. Over time, this starves the tissue of nutrients and oxygen. The outer coat then loses its strength, its collagen breaks down, and the wall thins like worn leather. Because the process can be “quiet,” patients may not feel much pain, even though the tissue is losing thickness. The risk is that a thin spot can bulge (a staphyloma) or rupture with little trauma, so detection and protection matter. EyeWiki

Types

1) By location (where the thinning is): Most thinning starts near the edge of the cornea (the limbus) and can spread in arcs. Doctors describe “peripheral” patches near the limbus and, less often, more central or broader arcs. The location matters because limbal areas face more mechanical stress from blinking.

2) By severity (how thin it is): Early disease may show subtle, gray or slate-blue patches where the darker inside of the eye shines through. Moderate disease shows translucent areas with visible underlying color. Severe disease can show frank “uveal show,” bulging, or areas at real risk for rupture.

3) By association (what it’s linked with): The classic association is long-standing rheumatoid arthritis. Other autoimmune conditions and a few infections can play a role. When an autoimmune disease is active in the body, it can fuel the eye problem even if the eye itself looks calm. EyeWikiAAO-HNS

---

Causes

1) Rheumatoid arthritis (most common link): Long-standing RA can damage small eye vessels and the sclera through immune complexes and vasculitis. Thinning tends to advance quietly, so people may not notice until the wall is very thin. AAO-HNSPMC

2) Granulomatosis with polyangiitis (ANCA-associated vasculitis): This disease inflames small blood vessels across the body and can target the eye’s feeding vessels, starving the sclera and leading to thin, bluish patches. EyeWiki

3) Microscopic polyangiitis (another ANCA-associated vasculitis): Similar to GPA, this condition can quietly reduce scleral blood flow and trigger melting, even when the eye does not look inflamed.

4) Relapsing polychondritis: Because this disease attacks collagen-rich tissues (like cartilage), it can also affect the collagen matrix of the sclera and cause quiet thinning in susceptible areas. AAO-HNS

5) Systemic lupus erythematosus: Lupus can form immune complexes that settle in small vessels, leading to poor perfusion and slow weakening of the sclera’s structure. EyeWiki

6) Polyarteritis nodosa: This medium-vessel vasculitis can produce ischemia in ocular tissues; long-term damage can present as scleral melt without redness. EyeWiki

7) Seronegative spondyloarthropathies (e.g., ankylosing spondylitis, psoriatic arthritis): These immune conditions sometimes involve the eye. Chronic, low-grade vascular injury may set the stage for scleral thinning. EyeWiki

8) Sjögren’s syndrome: Severe dryness and autoimmune activity can impair surface healing and, together with vasculitic mechanisms, contribute to scleral weakening in vulnerable patients.

9) Inflammatory bowel disease (ulcerative colitis/Crohn’s): Systemic inflammation and immune complex disease may extend to the sclera, producing slowly progressive thinning. EyeWiki

10) Limited scleroderma: Changes in microvasculature and collagen turnover can, in rare instances, contribute to scleral atrophy in quiet eyes. EyeWiki

11) Behçet disease: Although Behçet’s is best known for oral ulcers and uveitis, vascular inflammation can occasionally affect scleral health, leading to thinning. EyeWiki

12) Graft-versus-host disease (after transplant): Ocular surface disease and immune dysregulation can impair scleral support and healing, allowing “quiet” melt to develop. EyeWiki

13) Syphilis: A chronic infection that can mimic many diseases; syphilis can underlie scleritis and must be ruled out, because undiagnosed infection can worsen thinning and change treatment. EyeWiki

14) Tuberculosis: TB can trigger ocular inflammation or post-infectious immune reactions; identifying and treating TB is essential to prevent ongoing scleral damage. EyeWiki

15) Herpes zoster ophthalmicus: Zoster can injure ocular tissues and blood supply; in selected cases a quiet melt pattern may follow prior inflammation. EyeWiki

16) Lyme disease (less common): Chronic infection-related immune responses can involve the sclera; clinicians test for it when other clues point that way. WebEye

17) Gout/hyperuricemia: Uric acid problems can be associated with scleral disease; measuring uric acid is part of the workup because crystals and inflammation can harm ocular coats. EyeWiki

18) Surgically induced necrotizing scleritis history: Prior ocular surgery with tissue-toxic adjuncts (e.g., mitomycin C or beta-irradiation) can leave sclera vulnerable; later, a non-inflamed melt can resemble scleromalacia perforans. EyeWiki

19) Post-traumatic weakening: Old injuries or chemical exposures can thin sclera; years later a silent atrophic patch can enlarge even without obvious inflammation.

20) Idiopathic immune process: Sometimes no clear cause is found. Doctors still search carefully for hidden autoimmune or infectious triggers because catching them can prevent progression. EyeWiki

---

Symptoms

1) Painless irritation: People often describe a gritty or dry feeling rather than real pain. This mismatch is a clue because severe thinning can happen without classic pain. The Journal of Medical Optometry (JoMO)

2) Color change on the white of the eye: Family or the person themselves may notice a slate-gray or bluish area where the dark layer inside shows through a thinned wall. EyeWiki

3) Subtle vision blur over months: Vision may slowly change due to irregular astigmatism from bulging at thin spots, even when the eye looks calm. EyeWiki

4) Light sensitivity in bright sun: Even without redness, the eye can feel sensitive because the surface is stressed and the curvature is changing.

5) Mild foreign-body sensation: People may feel “something in the eye” from surface dryness over the thin area.

6) Trouble with contact lens comfort: A thinning, bulging area can make lenses less stable, creating blur or discomfort.

7) Changing glasses prescription: New or increasing astigmatism may show up during refraction as the wall bulges slightly. EyeWiki

8) Occasional redness nearby: The main patches are “quiet,” but other eye surfaces can still get mildly red from dryness or strain.

9) Episodes of ghosting/doubling: Irregular corneal shape overlying a thin scleral area can cause “shadow” edges around letters.

10) Achy feeling with eye movement (less common): True scleromalacia perforans is classically painless, but some patients still feel vague ache, especially if another scleritis pattern coexists. EyeWiki

11) Visible dark patch near the limbus: People may notice a dark crescent near the cornea edge, which is simply the uvea shining through.

12) Cosmetic concern: The color contrast can be alarming even when symptoms are mild.

13) Anxiety about eye “fragility”: Once told the wall is thin, people often fear bumping the eye; this fear is reasonable because thin walls can rupture with minor trauma. EyeWiki

14) Dryness that does not improve with usual drops: Surface instability over thin sclera may make simple lubricants feel less effective.

15) Rare sudden loss of vision (emergency): If a thin spot ruptures or if there is associated retinal detachment from coexisting posterior disease, vision can drop suddenly; this needs urgent care. PMC

---

Diagnostic tests

A) Physical exam (whole-person and eye-in-room checks)

1) General medical exam and joint check: The clinician looks for signs of rheumatoid arthritis or other autoimmune disease, such as hand joint swelling, nodules, skin rashes, or sinus disease. Eye and body findings together help point to the cause. EyeWiki

2) Blood pressure and vascular exam: Because vasculitides can involve many organs, checking vital signs and vessels helps spot systemic disease that might be harming ocular blood supply. EyeWiki

3) External eye inspection in natural light: Doctors study color and surface features. Scleromalacia perforans areas often look avascular and slate-blue without inflammation, which separates them from “red” painful scleritis. EyeWiki

4) Eyelid and tear-film assessment: Severe dryness, lid margin disease, or exposure can worsen surface stress over a thin wall, so the exam includes lids, blink, and tear stability.

5) Protective behavior and trauma risk review: Clinicians ask about eye rubbing, sports, or workplace hazards, because thin sclera is rupture-prone with minor bumps. EyeWiki

B) Manual tests (hands-on, low-tech clinic checks)

6) Best-corrected visual acuity (and pinhole): Measuring vision with and without pinhole helps tell if blur is from optics (like irregular astigmatism) versus other causes; progressive astigmatism is common as thin spots alter shape. EyeWiki

7) Refraction: A full refraction maps how glasses need to change. Shifts toward irregular astigmatism can track disease effect on shape.

8) Pupillary light reflex and swinging-flashlight test: This checks optic nerve function. It is usually normal in isolated anterior disease, which helps exclude other causes of vision loss.

9) Confrontation visual fields: A simple manual screen looks for field defects. If abnormal, it prompts imaging to exclude posterior involvement.

10) Gentle intraocular pressure measurement: Measuring pressure (with caution) helps detect bulging-related changes and avoids unduly pressing on thin areas; clinicians avoid any maneuver that could stress the fragile wall.

C) Slit-lamp biomicroscopy and bedside ocular tests

11) Slit-lamp exam of sclera and limbus: This is the cornerstone. Doctors look for avascular, thin, slate-blue patches; uveal show; and staphyloma. Quiet, white surroundings with prominent thinning favor scleromalacia perforans. EyeWiki

12) Red-free illumination and vessel mobility test: Deep scleral vessels don’t blanch with phenylephrine and are adherent; in scleromalacia perforans the hallmark is the lack of active vascular congestion and the striking avascular thin patch. EyeWiki

13) Fluorescein surface assessment (careful): Dye highlights surface defects. Clinicians avoid pressure or provocation in very thin areas, but surface staining patterns can reveal dryness over thin sclera and rule out actual leaks.

14) Seidel test for leakage (only if truly needed and with extreme care): If a focal area looks suspicious for a break, a Seidel test may be used to detect aqueous leak; however, because the wall is fragile, this is done gently and rarely.

D) Laboratory & pathological tests (to find the cause)

15) CBC with differential: Looks for signs of infection or inflammation and provides a baseline before any systemic treatment. Review of Optometry

16) ESR and/or CRP: These markers reflect systemic inflammatory activity; they can be normal in “quiet” eyes but still guide the underlying disease search. EyeWiki

17) Autoantibodies (RF and anti-CCP for RA; ANA for lupus; ANCA for vasculitis): These tests help uncover the major autoimmune culprits—especially RA and ANCA-associated vasculitis—that are classically tied to scleromalacia perforans. PMC

18) Syphilis serology (non-treponemal + treponemal tests): Syphilis can mimic or drive scleral disease, so doctors routinely rule it out. EyeWiki

19) TB testing (IGRA/PPD) and chest evaluation as indicated: TB can be an underlying driver; identifying it prevents inappropriate immunosuppression. EyeWiki

20) Serum uric acid and sarcoid screen as indicated: Uric acid problems and sarcoidosis show up in the scleritis workup lists and can orient the cause, especially if other clues match. EyeWiki

E) Imaging tests (to measure thickness and look behind the eye)

21) Anterior segment OCT (AS-OCT): This non-contact scan can quantify scleral thickness and monitor change over time in anterior scleritis, helping document thinning and response to therapy. It is particularly helpful because it is gentle on fragile eyes. PubMedPMC

22) Ultrasound biomicroscopy (UBM): UBM gives high-resolution images of anterior coats. It helps when OCT views are limited and can show the interface between the thin sclera and surrounding tissues.

23) Corneal topography/tomography: These maps reveal irregular astigmatism and shape changes that accompany bulging over thin sclera, explaining fluctuating vision and guiding optical correction.

24) B-scan ultrasonography (for suspected posterior involvement): If symptoms or exam suggest disease behind the eye, B-scan can show sclero-choroidal thickening, the classic “T-sign,” or fluid in Tenon’s space. This test is key when vision drops suddenly or pain suggests deeper disease. PMCPubMed

25) Orbital MRI (selected cases): MRI helps differentiate posterior scleritis from tumors or orbital inflammation and maps fluid and tissue changes that a slit-lamp cannot see. EyeWiki

Non-pharmacological treatments (Therapies & “others”)

Each item explains: description → purpose → how it works.

1. Urgent co-management with a rheumatologist — You see an eye specialist and a rheumatologist together. Purpose: align eye care with control of the underlying autoimmune disease. Mechanism: treating the whole-body immune driver (e.g., RA) reduces the immune attack on the sclera and lowers the risk of new melting. NCBI

2. Protective rigid eye shield (not a soft patch) during high-risk activities — A lightweight shield protects from bumps. Purpose: prevent trauma to the thinned wall. Mechanism: mechanical barrier while the wall is fragile.

3. Frequent preservative-free lubricating drops + nighttime ointment — Non-medicated tears and gel protect the surface. Purpose: maintain a smooth, moist surface and reduce friction from blinking. Mechanism: lowers microtrauma and inflammatory triggers on the ocular surface that can worsen adjacent melts. (Dry ocular surface often coexists in RA.)

4. Treat lid disease and ocular surface hygiene — Gentle lid scrubs/heat to control blepharitis/meibomian dysfunction. Purpose: calmer surface; fewer inflammatory mediators and enzymes that can travel to the sclera/cornea. Mechanism: reduces tear film instability and protease load that can aggravate melts. reviewofophthalmology.com

5. Moisture chamber goggles / humidified environment — Simple wraparound glasses or sleep masks hold humidity. Purpose: reduce evaporation and shear. Mechanism: improves tear film residence time, easing surface stress near thinned areas.

6. UV-blocking eyewear outdoors — Sunglasses with good side coverage. Purpose: cut phototoxic stress and ocular surface irritation. Mechanism: less UV-triggered surface inflammation.

7. Avoid eye rubbing and heavy straining (Valsalva) — Be mindful during coughing fits, lifting, or constipation. Purpose: reduce sudden pressure spikes and mechanical stress on thinned sclera. Mechanism: protects a wall that has little structural reserve.

8. Systemic health optimization (blood pressure, blood sugar, nutrition) — Work with primary care. Purpose: improve healing capacity and surgical outcomes. Mechanism: better collagen and tissue repair, lower infection risk.

9. Stop smoking — Smoking impairs wound healing. Purpose: support tissue repair. Mechanism: improves microvascular supply and fibroblast function.

10. Artificial tear “gels” before sleep and after waking — Thicker lubricants at vulnerable times. Purpose: reduce morning friction when lids open. Mechanism: decreases shear on thinned areas.

11. Cold preservative-free compresses for irritation (if advised) — Brief, clean, cooled pads. Purpose: symptom relief without steroids/NSAIDs if surface irritation exists. Mechanism: vasoconstriction and reduced mediator diffusion on the surface.

12. Nutritional pattern: anti-inflammatory diet — More marine omega-3s, high-color vegetables/fruit, lean protein; limit ultra-processed foods. Purpose: calm systemic inflammation that feeds eye disease. Mechanism: omega-3 derived lipid mediators compete with arachidonic pathways and can reduce RA activity. PMC+1

13. Vitamin D repletion if low — Check serum level with your clinician. Purpose: immune modulation and ocular surface comfort. Mechanism: vitamin D can improve dry eye symptoms and has anti-inflammatory effects. PMC+1ScienceDirect

14. Vitamin C adequacy from diet — Citrus, berries, peppers, greens. Purpose: collagen support. Mechanism: vitamin C is required for collagen cross-linking; deficiency weakens connective tissues (including sclera). NCBIEyeWiki

15. Careful contact lens policy (often avoid) — Standard bandage lenses can be risky over areas of extreme scleral/corneal thinning. Purpose: avoid pressure or micro-trauma. Mechanism: eliminates foreign-body friction on compromised tissue.

16. Infection vigilance — Prompt care for conjunctivitis or corneal ulcers. Purpose: infections add enzymes (MMPs) that worsen melting. Mechanism: faster control prevents protease-driven tissue loss. ScienceDirect

17. Medication review — Your team checks for drugs that can aggravate ocular surface (e.g., dryness) and weighs steroid risks/benefits. Purpose: reduce iatrogenic harm. Mechanism: tailor therapy to eye condition and systemic disease. NCBI

18. Safe exercise and joint-friendly activity — Gentle, regular movement. Purpose: improve systemic RA control and mood without eye strain or injury. Mechanism: anti-inflammatory effects of exercise.

19. Regular scheduled follow-ups with slit-lamp photos — Serial measurements and photos to track thinning. Purpose: catch small changes early. Mechanism: objective comparison over time. EyeWiki

20. Emergency plan — Know signs of sudden pain, gush of fluid, or vision drop and where to go 24/7. Purpose: rapid response if perforation occurs. Mechanism: time-sensitive globe-saving care.

---

Drug treatments

Key principle: SP almost always needs systemic immunosuppression because the disease is driven by a body-wide immune process. NSAIDs and eye drops are not enough if there is necrosis or progressive thinning. NCBI

1. Prednisone (systemic corticosteroid; bridge therapy) — Class: corticosteroid. Dose (typical adult): ~0.5–1 mg/kg/day (often 60–80 mg/day) short term, then taper. When: to rapidly quiet associated inflammatory activity or while steroid-sparing drugs begin to work. Purpose: urgent control of immune-mediated tissue destruction. Mechanism: broad suppression of cytokines and leukocyte trafficking. Side effects: high blood sugar, blood pressure, mood changes, infection risk, bone loss. NCBI

2. Methotrexate — Class: antimetabolite DMARD. Dose: ~0.15 mg/kg once weekly (commonly 7.5–25 mg/week) + folic acid. When: first-line steroid-sparing agent for noninfectious scleritis and RA. Purpose: long-term control of autoimmunity. Mechanism: inhibits folate-dependent pathways and down-regulates inflammatory cells. Side effects: liver enzyme elevation, cytopenias, mouth sores; avoid in pregnancy. NCBI

3. Azathioprine — Class: purine synthesis inhibitor. Dose: ~1–2 mg/kg/day. When: alternative steroid-sparing agent. Purpose: maintain remission and protect sclera. Mechanism: reduces lymphocyte proliferation. Side effects: leukopenia, liver toxicity; TPMT testing may guide safety. NCBI

4. Mycophenolate mofetil (MMF) — Class: inosine monophosphate dehydrogenase inhibitor. Dose: 1–3 g/day divided. When: steroid-sparing; useful when MTX or AZA are not tolerated. Mechanism: preferentially suppresses T- and B-cell proliferation. Side effects: GI upset, leukopenia, infection risk. NCBI

5. Cyclophosphamide — Class: alkylating agent. Dose: oral 1–2 mg/kg/day or IV pulses per rheumatology protocol. When: severe, vision-threatening necrotizing disease or vasculitis. Purpose: powerful control of aggressive autoimmunity. Mechanism: cross-links DNA, depleting rapidly dividing immune cells. Side effects: cytopenias, hemorrhagic cystitis, malignancy risk—specialist monitoring required. Oxford Academic

6. Rituximab — Class: anti-CD20 monoclonal antibody. Dose: 1,000 mg IV on day 1 and 15 (common RA schedule), repeat by response. When: refractory necrotizing scleritis or RA-linked disease. Purpose: deplete B cells that drive antibody-mediated damage. Mechanism: targets CD20 on B lymphocytes. Side effects: infusion reactions, infections; screen for hepatitis B. Evidence: case reports/series show control of necrotizing scleritis; sometimes combined with infliximab for rapid control. PMCPubMed

7. Infliximab — Class: anti-TNF-α monoclonal antibody. Dose: 3–5 mg/kg IV at weeks 0, 2, 6; then every 8 weeks (adjust per response). When: refractory cases or when steroid-sparing needed quickly. Mechanism: neutralizes TNF-α, a key cytokine in RA/ocular inflammation. Side effects: infections (TB reactivation), infusion reactions, demyelination risk. Evidence: used in severe scleritis; sometimes short-term with rituximab. PMC

8. Adalimumab — Class: anti-TNF-α monoclonal antibody. Dose: 40 mg SC every 2 weeks (RA schedule). When: alternative anti-TNF for refractory disease under specialist care. Mechanism/risks: as above for TNF blockade. Note: evidence includes broader scleritis/uveitis literature and RA control paradigms. NCBI

9. Tocilizumab — Class: anti-IL-6 receptor monoclonal antibody. Dose: 8 mg/kg IV monthly or 162 mg SC weekly/biweekly. When: refractory disease, RA-linked. Mechanism: blocks IL-6 pathway to reduce stromal destruction. Side effects: infection risk, LFT elevation, lipids. (Supported by noninfectious ocular inflammation experience.) SpringerLink

10. Doxycycline (adjunct) — Class: tetracycline antibiotic with MMP-inhibiting effect. Dose: 50–100 mg twice daily (adjunct dosing). When: as a helper to reduce corneal/scleral melt enzymes and stabilize the surface. Mechanism: inhibits matrix metalloproteinases and down-regulates IL-1; can slow collagen breakdown. Side effects: GI upset, photosensitivity; avoid in pregnancy/children. PubMedreviewofophthalmology.com

Important: Dosing varies with body weight, comorbidities, and lab monitoring; your clinicians will tailor regimens and screen for infection or vasculitis before escalating therapy.

---

Dietary “molecular” supplements

Evidence for supplements in SP specifically is limited; choices below target systemic inflammation (e.g., RA) or tissue health. Always discuss with your clinician to avoid drug interactions.

1. Omega-3 EPA/DHA — Dose: ~2–3 g/day EPA+DHA. Function: systemic anti-inflammatory support; may reduce RA pain and stiffness. Mechanism: shifts eicosanoid balance to pro-resolving mediators. PMC+1

2. Vitamin D3 (cholecalciferol) — Dose: often 1,000–2,000 IU/day (or per lab-guided repletion). Function: immune modulation; may improve dry-eye comfort. Mechanism: vitamin D receptors dampen inflammatory signaling. PMC+1

3. Vitamin C — Dose: 200–500 mg/day (from diet preferred). Function: collagen synthesis cofactor. Mechanism: pro-collagen hydroxylation strengthens connective tissue. (Deficiency weakens tissues.) NCBI

4. Curcumin (turmeric extract, standardized) — Dose: 500–1,000 mg twice daily, often with bioavailability enhancer (e.g., piperine—check drug interactions). Function: adjunct anti-inflammatory for arthritis symptoms. Mechanism: NF-κB and cytokine modulation. PMCFrontiers

5. N-acetylcysteine (NAC) — Dose: 600 mg twice daily. Function: antioxidant/anti-inflammatory support; ocular surface healing data exist for topical and systemic forms in other eye diseases. Mechanism: replenishes glutathione; reduces oxidative stress. PMCScienceDirect

6. Lutein + Zeaxanthin — Dose: 10 mg + 2 mg/day. Function: general ocular antioxidant support. Mechanism: quenches reactive oxygen species in ocular tissues.

7. Green-tea catechins (EGCG) — Dose: often 300–400 mg/day (from tea/extracts; avoid high doses with liver disease). Function: anti-inflammatory/antioxidant adjunct. Mechanism: inhibits NF-κB and oxidative cascades.

8. Coenzyme Q10 — Dose: 100–200 mg/day. Function: mitochondrial antioxidant support; potential aid in fatigue with chronic inflammation. Mechanism: electron transport cofactor; reduces oxidative injury.

9. Zinc (moderate dose) — Dose: 15–25 mg/day unless otherwise directed. Function: wound-healing enzyme cofactor; avoid high doses long-term. Mechanism: supports collagen cross-linking enzymes.

10. Probiotics (strain-specific, evidence evolving) — Dose: per product. Function: gut-immune axis modulation in RA adjunct care. Mechanism: microbiome effects on systemic cytokines. ScienceDirect

---

Regenerative” therapies

These target the immune root cause. “Stem-cell” approaches remain experimental for RA/ocular indications; the biologics below are the current evidence-based options.

1. Rituximab — Dose: 1,000 mg IV day 1 & 15 (RA schedule). Function: B-cell depletion for refractory necrotizing scleritis. Mechanism: anti-CD20 monoclonal antibody. Notes: supported by case series/reports in severe scleritis. PMC

2. Infliximab — Dose: 3–5 mg/kg IV at 0, 2, 6 weeks then q8w. Function: rapid cytokine blockade. Mechanism: anti-TNF-α. Notes: used as a bridge or combined strategy in aggressive disease. PMC

3. Adalimumab — Dose: 40 mg SC q2w. Function/Mechanism: TNF-α inhibition for steroid-sparing control in RA-linked ocular inflammation. NCBI

4. Tocilizumab — Dose: 8 mg/kg IV monthly or 162 mg SC. Function: IL-6 pathway blockade. Mechanism: reduces inflammatory signaling that drives stromal destruction. SpringerLink

5. Abatacept — Dose: weight-based IV or 125 mg SC weekly (RA). Function: T-cell costimulation blocker (CTLA4-Ig). Mechanism: interrupts CD80/86–CD28 signaling; dampens autoimmune activity affecting the sclera. (Evidence from RA and ocular inflammation experience.) NCBI

6. IVIG (intravenous immunoglobulin) — Dose: often 2 g/kg split over 2–5 days monthly (specialist protocols). Function: immunomodulation where other agents fail or are contraindicated. Mechanism: Fc-mediated immune regulation.

About “stem-cell drugs”: There are no approved stem-cell medications for SP. Mesenchymal stem-cell (MSC) therapy is under study for autoimmune diseases like RA; early studies suggest safety and possible benefit, but high-quality trials are still needed before routine clinical use. For the eye, stem-cell therapy mainly targets corneal/limbal disorders, not scleral melting. Discuss clinical trials only within regulated centers. PLOSPMC+1

---

Surgeries

1. Scleral patch graft — A donor scleral plate is sutured over the thinned area; often covered with conjunctiva or amniotic membrane. Why: to restore tectonic strength and prevent rupture. Evidence: effective for preserving globe integrity; preserved sclera plus amniotic membrane is a simple, fast option in large defects. NaturePubMed

2. Conjunctival advancement (pedicle) flap ± Tenon’s — Vascularized conjunctiva is advanced to cover exposed tissue. Why: provides biologic cover, blood supply, and protection when donor tissue is limited or as a temporizing measure. (More commonly used for corneal melts/perforations, but principles apply for surface coverage.) PMCNature

3. Tectonic corneoscleral (sclerokeratoplasty) or corneal lamellar graft — Larger graft replacing diseased cornea/sclera in extensive melts. Why: to remove necrotic tissue and rebuild the wall when involvement extends onto the cornea. PMCNature

4. Cyanoacrylate tissue adhesive (glue) with bandage protection — For small, focal perforations, glue seals the leak; often a temporary step before definitive grafting. Why: immediate closure to save the eye. PMCAAO

5. Autologous fascia lata or other biomaterial grafts (select cases) — Using patient’s own tissue or alternatives like dermis, periosteum, cartilage, or synthetic Gore-Tex® when scleral tissue is unsuitable. Why: versatility when standard scleral graft is not possible. (Historic and modern reports support the approach.) PubMedEyeWiki

---

Practical prevention tips

1. Keep RA and any vasculitis tightly controlled with your rheumatologist.

2. Never ignore a new gray/dark “window” or bulge on the white of the eye.

3. Use protective eyewear at work, play, and during home projects.

4. Maintain surface comfort (lubricants, hygiene) to reduce melt-driving enzymes. reviewofophthalmology.com

5. Stop smoking and optimize sleep, nutrition, and exercise for healing.

6. Avoid eye rubbing and minimize pressure spikes (treat cough/constipation).

7. Keep a medication list; review corticosteroid/NSAID plans with your doctors. NCBI

8. Treat infections promptly; report any red, painful, or sticky eye.

9. Attend scheduled follow-ups even if the eye feels fine—SP can be silent. EyeWiki

10. Have an emergency plan for sudden pain, fluid leakage, or vision loss.

---

When to see a doctor—right away

• You notice a new dark/gray patch, a bulge, or a step-off in the white of the eye.

• Sudden pain, tearing/leak, or a feeling that the eye is softer.

• Rapid vision change, new redness, light sensitivity, or double vision.

• You have RA/autoimmune disease and any new eye symptom—even if the eye looks white. (SP can be painless until advanced.) EyeWiki

---

What to eat more of—and what to avoid

1. Eat: fatty fish (salmon, sardines) 2–3×/week or fish-oil per clinician (EPA/DHA). Avoid: frequent ultra-processed, high-omega-6 snack foods. PMC

2. Eat: leafy greens, citrus, berries, bell peppers (vitamin C). Avoid: nutrient-poor sugary drinks that displace real food. NCBI

3. Eat: vitamin-D–rich foods (fortified dairy/alternatives, eggs) and supplement if low. Avoid: guessing—check a blood level first. PMC

4. Eat: spices like turmeric (curcumin) in cooking; discuss standardized supplements if desired. Avoid: unregulated high-dose blends that interact with meds. PMC

5. Eat: adequate lean protein (supports collagen repair). Avoid: excessive alcohol that impairs healing.

6. Eat: nuts, seeds, avocado, olive oil for healthy fats. Avoid: trans fats.

7. Drink: water regularly; consider green tea for polyphenols. Avoid: excess energy drinks.

8. Add: colorful vegetables daily (antioxidants). Avoid: crash diets that risk micronutrient deficiency.

9. Consider: NAC, lutein/zeaxanthin after clinician review. Avoid: “megadose” zinc or vitamin A. PMC

10. Keep it consistent: diet helps systemic inflammation; it doesn’t replace immunosuppressive therapy for SP.

---

Frequently Asked Questions

1. Is scleromalacia perforans the same as ordinary scleritis?
   No. It is a necrotizing form without obvious inflammation, often painless but more dangerous because thinning progresses silently. EyeWiki

2. Why does it happen?
   Usually because of an autoimmune attack (often RA) on small scleral vessels and the surrounding collagen, which leads to tissue death and thinning. EyeWiki

3. Can eye drops fix it?
   No. Systemic therapy is needed to stop the immune driver. Eye drops help comfort but do not halt necrosis. NCBI

4. Is it painful?
   Often no, which is why it can be missed. The eye may look white despite serious thinning. EyeWiki

5. How is it diagnosed?
   By slit-lamp exam showing avascular, thin sclera with uveal show-through; doctors also run blood tests and imaging to check for systemic disease. NCBI

6. What is the risk?
   Perforation and permanent vision loss if untreated; risk is higher with active systemic autoimmune disease. EyeWiki

7. Which medicines work best?
   Regimens that control the underlying autoimmunity—commonly methotrexate, azathioprine, mycophenolate, and, if severe or refractory, cyclophosphamide or biologics like rituximab or anti-TNF agents. Your team picks the safest effective option. NCBI

8. Do antibiotics help?
   Only if there is an infection; otherwise, antibiotics are not the main treatment (except doxycycline as an MMP-inhibiting adjunct). PubMed

9. Will I need surgery?
   Surgery is needed when there is exposed uvea, imminent rupture, or perforation, to reinforce the wall (e.g., scleral patch graft). Medical therapy continues to control the cause. Nature

10. Are there stem-cell cures?
    Not for SP today. Stem-cell/MSC approaches for RA and ocular surface are experimental and should only be considered in regulated trials. PLOS

11. Can this affect both eyes?
    Yes, especially with systemic disease—both eyes need monitoring. EyeWiki

12. Is it contagious?
    No. It is an autoimmune problem, not an infection. NCBI

13. Can glasses or contacts fix it?
    Glasses protect; standard contact lenses are usually not used over thin areas because they can rub and worsen the problem.

14. How long will treatment last?
    Immunosuppression is often long-term to prevent relapse; tapering too fast can risk re-activation. Plans are individualized.

15. What is the outlook?
    With early systemic therapy and surgical reinforcement when needed, many patients keep useful vision; delayed care raises the risk of perforation and vision loss. Nature

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: August 24, 2025.