Keratoendotheliitis Fugax Hereditaria (KEFH)

KFH is a rare, inherited eye problem that comes in attacks. An attack makes one eye suddenly red, painful, and very sensitive to light. The clear window of the eye (the cornea) gets a little swollen and hazy, so vision goes blurry for a few days. Then it calms down and the eye looks normal again. Over many years, some people can develop a faint scar in the center of the cornea that can slightly reduce vision. KFH runs in families in an autosomal dominant way, meaning a child has a 50% chance to inherit it if one parent has it. Research shows it is linked to a change (mutation) in a gene called NLRP3, which controls part of the body’s “inflammasome,” a switch that turns inflammation on and off. In KFH, that switch is too eager to turn on. NCBIPubMedPMC

Keratoendotheliitis Fugax Hereditaria (KEFH) is a rare, inherited eye condition that causes short, sudden attacks of inflammation in the clear front window of the eye (the cornea). During an attack, one eye (sometimes both, but usually one at a time) becomes red, painful, light-sensitive, watery, and blurry. The cornea swells (called edema), and your vision can look misty or foggy. These attacks are temporary—they typically last 2–5 days and then settle. In some people, repeated attacks over many years can leave faint central corneal scars that may slightly reduce vision between episodes. KEFH runs in families in an autosomal dominant way, meaning a child has a 50% chance of inheriting it if one parent is affected. NCBI

Scientifically, KEFH has been linked to a small change (a missense mutation, c.61G>C; p.Asp21His) in a gene called NLRP3. This gene helps control a “danger sensor” inside cells called the inflammasome. When this sensor is too easy to trigger, inflammation flares in the cornea’s inner lining (the endothelium), causing the short-lived attacks. KEFH belongs to the same broad family as cryopyrin-associated periodic syndromes (CAPS), but its inflammation is limited to the eye. PubMeddisorders.eyes.arizona.eduNCBIJACI

Typical attacks last 2–5 days, usually happen several times per year, and most often affect one eye at a time. Between attacks the eye often looks and feels normal. EyeWiki

The inflamed layer is mainly the corneal endothelium (the very thin inner cell layer that keeps the cornea clear and dehydrated). During a flare, eye doctors can see tiny dark patches on this layer (called pseudoguttae) and mild swelling; between attacks the endothelium may look normal. With repeated attacks over years, some people develop a central, oval stromal opacity (a faint, oval “film” inside the cornea). ScienceDirect+1EyeWiki

---

Types

There isn’t an official worldwide subtype system. Clinicians describe patterns that are helpful in practice:

1. By laterality

• Classic unilateral: one eye at a time (most common).

• Alternating bilateral: one eye, then the other in different attacks.

• Simultaneous bilateral: both eyes at once (less common).
  These patterns reflect how attacks appear; the underlying disease is the same. NCBI

2. By phase

• Acute phase (the attack): pain, light sensitivity, redness, corneal swelling/haze for 2–5 days.

• Quiet phase: eye looks normal again.

• Chronic footprint: in some adults after many attacks, a faint central stromal opacity remains. EyeWikiScienceDirect

3. By presence of central opacity

• Without central opacity (common earlier in life).

• With central, horizontally oval stromal opacity (reported in about ~47% in one large series). ScienceDirect

4. By severity over the lifespan

• Attacks often begin in childhood–young adulthood and tend to become less frequent and milder in middle age. Wikipedia

---

Causes

Important: The root cause is genetic. Many triggers have been reported by patients and studies. Some items below are proven; some are commonly reported or biologically plausible. I’ll label them clearly.

Root genetic mechanism (well established):

1. NLRP3 gene mutation (often p.Asp21His, c.61G>C) → overactive inflammasome → extra IL-1β inflammatory signaling in the cornea. (Proven.) PubMedPMC

2. Autosomal dominant inheritance → 50% chance to pass to children. (Proven.) NCBI

3. Other NLRP3 variants reported (rare families beyond the classic Finnish mutation). (Proven.) IOVS

Commonly reported attack triggers (observational/patient-reported):

1. Cold wind or cold air exposure. (Well reported.) ajo.com+1
2. Psychological stress. (Well reported.) ajo.com
3. Lack of sleep / fatigue. (Reported.) Wiley Online Library
4. Minor eye micro-trauma (e.g., rubbing) or injury. (Reported.) Wiley Online Libraryajo.com
5. Concurrent infections/illness (reported in some). Wiley Online Library
6. Windy, harsh weather (beyond temperature alone). (Reported together with cold exposure.) Helda

Disease behavior and modifiers (supported by cohort-level observations):

1. Age (attacks often start in childhood/teens; severity tends to fade later). Wikipedia
2. Frequency of past attacks (more total attacks over years may increase risk of central scarring). EyeWiki
3. Individual NLRP3 variant (some variants may influence phenotype; evidence is emerging). IOVS

Biologic contributors (inferred from CAPS biology; helpful to understand mechanism, but not direct “triggers”):

1. Overactive NLRP3 inflammasome priming in corneal cells. (Mechanistic.) Frontiers
2. Excess IL-1β signaling driving episodic corneal inflammation. (Mechanistic.) PMC
3. Cold-related protein aggregation heightening NLRP3 activity (seen in cryopyrin diseases). (Mechanistic, plausible link.) eLife

Other context (what doesn’t seem to be the cause):

1. Not a systemic CAPS illness (KFH has no systemic symptoms in typical cases). (Observed.) Genetic Eye Diseases Database
2. Not an eye infection (viruses like HSV/CMV can mimic this, but KFH itself is genetic and sterile inflammation). (Observed; differential.) SpringerLink

Family/epidemiology notes:

1. Family history in European ancestry (many reported families come from Finland; cases likely underrecognized elsewhere). (Observed.) Wikipedia
2. Penetrance within families (some members have more, some fewer attacks—typical for autosomal dominant traits). (General genetic principle; KFH cohorts show variability.) ScienceDirect
3. Unknown/idiopathic factors still exist (even with the same mutation, triggers and severity differ). (Observed variability.) ScienceDirect

---

Common symptoms

1. Eye pain that comes on suddenly (usually one eye). EyeWiki

2. Redness of the affected eye. EyeWiki

3. Photophobia (light hurts). EyeWiki

4. Blurred vision or a foggy spot. EyeWiki

5. Halos around lights during a flare (from corneal swelling). NCBI

6. Watering/tearing (epiphora). NCBI

7. Gritty/foreign-body sensation. NCBI

8. Mild double vision (rare; from blur/halos). NCBI

9. Headache during attacks (reported by ~40% in one series). ResearchGate

10. Decreased best-corrected vision during the attack; it improves as swelling clears. EyeWiki

11. Attacks last 2–5 days, with blurred vision sometimes lingering a bit longer. EyeWiki

12. 1–8 attacks per year on average (varies widely). EyeWiki

13. Usually one eye at a time; may alternate between eyes; rarely both at once. NCBI

14. Between attacks the eye often looks normal. Adults who’ve had many flares may have a faint central opacity. EyeWiki

15. No body-wide symptoms (no fever, joint pain, rash, etc.—unlike classic CAPS syndromes). Genetic Eye Diseases Database

---

20 Diagnostic tests (grouped and explained in plain English)

A) Physical exam and chairside checks

1. History of attacks — short bouts (2–5 days) of pain/redness/blurry vision in one eye, often since childhood or teens, sometimes with family history. This pattern is a big clue. EyeWikiWikipedia

2. Visual acuity — measures how much the attack reduces clarity. Improves as swelling settles. EyeWiki

3. Slit-lamp biomicroscopy — doctor looks closely at the cornea: finds pseudoguttae (dark patches at the endothelial layer), mild stromal haze, and slight swelling during a flare. Between attacks, the cornea may look normal. ScienceDirect

4. Anterior chamber check — sometimes there’s a mild cell/flare (tiny inflammatory cells/protein) during an attack. ScienceDirect

5. Intraocular pressure (IOP) — usually normal, but measured to help rule out Posner–Schlossman or other mimics that raise IOP during attacks. NCBI

B) “Manual” clinical tests (simple office procedures)

6. Fluorescein staining of the corneal surface — usually normal in KFH (the problem is inside the cornea), which helps rule out epithelial erosions/ulcers. (Differential logic.) SpringerLink

7. Corneal sensitivity (cotton-wisp) — normal in KFH; reduced sensation points more toward herpetic disease. (Differential logic for HSV.) EyeWiki

8. Gonioscopy — looks at the drainage angle to help exclude glaucomatocyclitic crisis (Posner–Schlossman). (Differential.) NCBI

9. Pinhole test — if vision improves through a pinhole, blurring is optical (from corneal haze/swelling) rather than nerve/retina. (General clinical principle; applied here.) EyeWiki

10. Over-refraction / trial with rigid or scleral lens (in clinic) — sometimes demonstrates how much a central stromal opacity limits vision and whether specialty lenses might help. (Recommended for reduced vision in KFH cohorts.) ResearchGate

C) Laboratory & pathological tests (to confirm KFH or rule out mimics)

11. Genetic testing for NLRP3 (e.g., c.61G>C, p.Asp21His) — confirms the KFH-type cryopyrin change. (Strong confirmatory test when available.) PubMed

12. Aqueous humor PCR for HSV (and often 13) CMV / 14) VZV) — not positive in KFH, but useful to exclude viral endotheliitis that can look similar and needs antivirals. EyeWiki

13. Histopathology (rare; e.g., if someone had a corneal transplant for another reason) — case series show inflammatory cell clusters in stroma that help explain the “pseudoguttae-like” look. ajo.com

D) Imaging / instrumented corneal tests

16. Specular microscopy — during flares shows large dark “holes” (pseudoguttae) in the endothelial mosaic; these resolve after attacks. ScienceDirect

17. In vivo confocal microscopy (IVCM) — during attacks shows hyper-reflective inflammatory cell clusters in the stroma; explains the endothelial “masking” effect. ScienceDirect

18. Pachymetry (ultrasound/optical central thickness) — thicker during attacks (fluid in cornea). Values trend back toward baseline after. ScienceDirect

19. Corneal topography/tomography (e.g., Scheimpflug/Pentacam) — may show irregular patterns and helps document/monitor central oval opacity if present. ScienceDirect

20. Serial slit-lamp photos — simple but powerful for tracking frequency and residual scarring over time; widely used in KFH studies and clinics. (Documentation standard; complements the items above.) EyeWiki

Non-pharmacological treatments

These are supportive measures you can start at home. They do not replace medical care. Start medical therapy early in a flare as your clinician recommends.

1. Attack action plan
   What: Keep a written “if-then” plan (what to start, who to call).
   Purpose: Faster, calmer response.
   How it works: Reduces treatment delay—important because early treatment shortens attacks. EyeWiki

2. Avoid cold wind exposure
   What: Use wrap-around glasses, scarf, or face shield in wind/cold.
   Purpose: Reduce trigger exposure.
   How: Blocks cold air that can provoke attacks in many patients. PubMedhelda.helsinki.fi

3. Stress-reduction routine
   What: Brief daily breathing, mindfulness, or stretching.
   Purpose: Lower stress-related flares.
   How: Damps sympathetic arousal that patients often link with episodes. PMC

4. Dark room + sunglasses
   What: Rest in dim light; wear sunglasses outdoors.
   Purpose: Ease photophobia and glare.
   How: Cuts light scatter across edematous cornea.

5. Cold compress (clean, not directly on the eye)
   What: 10 minutes on/10 off, several times a day.
   Purpose: Soothes pain and redness.
   How: Mild vasoconstriction may reduce surface discomfort.

6. Avoid eye rubbing
   Purpose: Prevents extra micro-trauma and epithelial irritation during a flare.
   How: Less mechanical stress on cornea.

7. Stop contact lens wear during attacks
   Purpose: Lowers irritation and infection risk; allows cornea to recover.
   How: Removes hypoxic/mechanical stress.

8. Eye shield at night
   Purpose: Prevents accidental rubbing while asleep.
   How: Physical barrier during the most vulnerable hours.

9. Humidifier and blink breaks
   Purpose: Support tear film and comfort, especially at screens.
   How: Moist air + frequent blinking reduces surface dryness that can worsen symptoms.

10. Work/school accommodations
    What: Temporary light duty, larger fonts, flexible deadlines.
    Purpose: Maintain function during flares.
    How: Reduces strain until edema clears (usually within days). NCBI

11. Driving precautions
    What: Don’t drive if vision is foggy.
    Purpose: Safety.
    How: Attacks can blur vision for 2–5 days. NCBI

12. Protective eyewear in dusty/ windy environments
    Purpose: Lower irritation and micro-trauma.
    How: Physical barrier.

13. Allergen/environmental control
    What: Reduce smoke, dust, strong fumes.
    Purpose: Less surface irritation during recovery.
    How: Limits reflex tearing and rubbing.

14. Hydration + frequent small sips
    Purpose: General comfort; supports tear film.
    How: Mild dehydration worsens ocular surface dryness.

15. Sleep optimization (7–9 h, regular schedule)
    Purpose: Promotes immune balance and pain tolerance.
    How: Better parasympathetic tone reduces stress reactivity.

16. Attack diary
    What: Note date, weather, stress, activities, response to meds.
    Purpose: Learn personal triggers/patterns.
    How: Guides prevention (e.g., more wind protection on risky days).

17. Early clinic contact policy
    What: Call promptly at first symptoms.
    Purpose: Early anti-inflammatory therapy can shorten attacks.
    How: Reduces cumulative scarring risk over years. EyeWiki

18. Family awareness + genetic counseling
    Purpose: Earlier recognition in relatives; informed planning.
    How: KEFH is autosomal dominant; counseling helps families prepare. NCBI

19. Avoid unnecessary topical anesthetics
    Purpose: Prevent toxicity and delayed healing.
    How: These are for in-office use only.

20. Regular specialist follow-up
    Purpose: Track subtle scarring and endothelial health.
    How: Monitoring helps adjust treatment and protect long-term vision. NCBI

Drug treatment

Important: Exact dosing and tapering are individualized. KEFH lacks large trials; recommendations are adapted from corneal inflammation care and case reports/series. Start and adjust only with your eye specialist. EyeWikiNCBI

1. Prednisolone acetate 1% eye drops (topical corticosteroid)
   Dosing window: often 1 drop 3–6 times/day for a few days, then short taper as directed.
   Purpose: Quell the acute inflammatory burst.
   Mechanism: Blocks many inflammatory signals (down-regulates inflammasome-driven cytokines).
   Side effects: Temporary eye-pressure rise (steroid response), delayed healing, rare infection risk with prolonged use. EyeWiki

2. Loteprednol etabonate 0.5% (soft steroid)
   Dosing: similar short course; often chosen when pressure risk is a concern.
   Purpose/Mechanism: Anti-inflammatory with lower average IOP effect.
   Side effects: Mild burning, possible IOP rise (less frequent than stronger steroids).

3. Dexamethasone 0.1%
   Dosing: short course if stronger effect needed; taper per clinician.
   Purpose: Potent inflammation control.
   Side effects: Higher IOP rise risk; use is clinician-guided.

4. Cyclopentolate 1% (cycloplegic)
   Dosing: 1 drop BID–TID during painful phase.
   Purpose: Reduces ciliary spasm–related pain and photophobia.
   Mechanism: Temporarily relaxes the ciliary muscle and dilates the pupil.
   Side effects: Blurry near vision, light sensitivity, rarely precipitates angle closure in predisposed eyes.

5. Hypertonic saline 5% drops / 6% ointment
   Dosing: drops QID and ointment HS during edema; may use briefly after.
   Purpose: Draw fluid out of the cornea to clear haze faster.
   Mechanism: Osmotic gradient pulls water from swollen corneal layers.
   Side effects: Stinging on instillation.

6. Lubricating tears (preservative-free carboxymethylcellulose/hyaluronate)
   Dosing: as needed (e.g., QID–Q2H during symptoms).
   Purpose: Comfort, smoother optics while edema settles.
   Mechanism: Supplements tear film, reduces friction; does not treat the core inflammation.
   Side effects: Minimal (choose preservative-free for frequent use).

7. Topical NSAID (e.g., ketorolac 0.5%)—use cautiously
   Dosing: short course if prescribed.
   Purpose: Additional pain control.
   Mechanism: Blocks prostaglandins.
   Side effects: Can delay epithelial healing or cause irritation; many cornea specialists prefer oral NSAIDs instead of topical for this reason. Use only if your doctor recommends. EyeWiki

8. Oral NSAID (e.g., ibuprofen 200–400 mg every 6–8 h with food)
   Purpose: Pain relief; some patients report better symptom control than with drops alone.
   Mechanism: Systemic prostaglandin reduction.
   Side effects: Stomach upset, kidney effects with overuse, drug interactions. disorders.eyes.arizona.edu

9. Oral antihistamine (e.g., cetirizine 10 mg once daily)
   Purpose: Symptom relief, sedation at night may help comfort.
   Mechanism: Histamine receptor blockade; does not treat the endothelial inflammation itself.
   Side effects: Dry mouth, drowsiness (varies by product). EyeWiki

10. Oral analgesic (e.g., acetaminophen 500–1,000 mg up to Q6–8h; max per label)
    Purpose: Pain control if NSAIDs are unsuitable.
    Mechanism: Central analgesia.
    Side effects: Liver toxicity if overdosed; heed label limits.

Evidence anchor: Reviews and reference texts describe short courses of topical steroids as first-line, with supportive agents (cycloplegic, hypertonic saline, analgesics) as needed; oral NSAIDs/antihistamines can help symptoms. There are no randomized trials specific to KEFH. NCBIEyeWiki

Regenerative” options

These are not established treatments for KEFH. They are listed for completeness because KEFH is linked to the NLRP3/IL-1 pathway and corneal endothelial recovery research is rapidly evolving. Consider only in clinical trials or specialist guidance.

1. Anakinra (IL-1 receptor antagonist, injection)
   Dose used in CAPS: often 100 mg subcutaneously daily (varies).
   Rationale: KEFH shares the NLRP3/IL-1 axis with CAPS; IL-1 blockade helps CAPS.
   Status in KEFH: No published KEFH trials; theoretical benefit only.
   Risks: Injection reactions, infection risk. JACI

2. Canakinumab (anti–IL-1β monoclonal antibody, injection)
   CAPS dosing (adult): typically 150 mg SC every 8 weeks (weight-based in children).
   Rationale/Status/Risks: As above—theoretical for KEFH; infection risk; costly. JACI

3. Rilonacept (IL-1 trap, injection)
   Rationale/Status: Targets IL-1β/IL-1α signaling; no KEFH data.
   Risks: Injection site reactions, infection risk. JACI

4. ROCK inhibitors (ripasudil 0.4% in Japan; netarsudil 0.02%)—off-label for endothelial recovery
   Dose used in studies: ripasudil often QID; netarsudil often QD.
   Purpose: Promote corneal endothelial cell migration/proliferation and speed clearing after endothelial injury in other conditions.
   Status for KEFH: Investigational/off-label; may be considered only with specialist guidance.
   Risks: Redness; netarsudil can cause reticular epithelial edema in some corneas. PubMed+1PMCScienceDirect

5. Cultured human corneal endothelial cell (hCEC) therapy with ROCK inhibitor (e.g., Y-27632)
   What it is: Injection of lab-grown human endothelial cells with a ROCK inhibitor to repopulate the endothelium (studied for corneal edema from other causes).
   Purpose: Regenerate/restore endothelial function.
   Status: Early trials show multi-year safety and efficacy for endothelial failure, not KEFH; not standard for KEFH.
   Risks: Surgical/immune risks, availability limited to trials or specialized centers. New England Journal of MedicinePubMedLippincott Journals

6. Experimental NLRP3 inflammasome inhibitors (e.g., MCC950, β-hydroxybutyrate, resveratrol, miR-223 agonism)
   Form: oral/systemic or topical strategies under investigation (not approved for KEFH).
   Purpose: Directly damp NLRP3 inflammasome signaling.
   Status: Mechanistic/early ocular studies only; no clinical KEFH trials.
   Risks: Unknown/variable; use only in research settings. NCBI

Surgeries

1. Phototherapeutic keratectomy (PTK)
   What: Excimer laser gently polishes superficial corneal haze.
   Why: If repeated attacks leave superficial central scars that blur vision.
   Note: Helps surface opacities; not for deep endothelial dysfunction.

2. Superficial keratectomy (manual polish)
   What: Surgeon manually removes superficial scar tissue.
   Why: Alternative to PTK when suited to the scar’s depth/shape.

3. Descemet Membrane Endothelial Keratoplasty (DMEK)
   What: Microsurgery replacing only the diseased corneal endothelium.
   Why: For rare cases with persistent endothelial failure and edema not clearing between attacks.

4. DSAEK (endothelial lamellar transplant)
   What: Slightly thicker endothelial-backing transplant than DMEK.
   Why: Similar indication; surgeon preference/anatomy guide choice.

5. Penetrating keratoplasty (full-thickness corneal transplant)
   What: Full corneal replacement.
   Why: Reserved for dense scarring or combined problems unfixable by lamellar options.

Most KEFH patients never need surgery. Attacks are brief; the aim is to control flares early and minimize cumulative scarring over time. NCBI

Prevention pointers

• Shield from cold wind (wrap-around eyewear, scarf). PubMed

• Manage stress (daily 5–10 minutes of relaxation). PMC

• Don’t rub eyes—especially during attacks.

• Stop contact lenses during symptoms.

• Keep an attack action plan (early steroid/cycloplegic as prescribed). EyeWiki

• Maintain regular follow-ups for endothelial checks. NCBI

• Control environmental irritants (smoke, dust).

• Use protective eyewear in harsh weather/at work.

• Educate family (autosomal dominant inheritance). NCBI

• Seek care promptly if an episode doesn’t improve in 24–48 hours or new symptoms (severe pain, marked light sensitivity) arise. NCBI

Dietary / molecular and supportive supplements

There’s no supplement proven to prevent KEFH attacks. Some nutrients affect general ocular surface comfort or inflammasome biology. Discuss with your clinician, especially if pregnant, on blood thinners, or with medical conditions. Evidence for KEFH specifically is absent; notes below use broader ocular/inflammatory science, and in some cases, NLRP3-targeting lab data. NCBI

1. Omega-3s (EPA/DHA; e.g., 1,000–2,000 mg/day)
   Function: anti-inflammatory lipid mediators; support tear film.
   Mechanism: shifts eicosanoids toward less inflammatory pathways.

2. Vitamin C (e.g., 500 mg/day)
   Function: antioxidant support for collagen/epithelium.
   Mechanism: quenches reactive oxygen species during inflammation.

3. Vitamin D (per labs; often 800–2,000 IU/day if low)
   Function: immune modulation.
   Mechanism: nuclear receptor effects on innate immunity.

4. Resveratrol (e.g., 100–250 mg/day)
   Function: antioxidant; cited as NLRP3-modulating in ocular literature.
   Mechanism: down-tunes inflammasome activation (preclinical). EyeWiki

5. Curcumin (e.g., 500–1,000 mg/day, with pepper or formulated for absorption)
   Function: general anti-inflammatory.
   Mechanism: inhibits NF-κB pathways upstream of inflammasome.

6. Quercetin (e.g., 250–500 mg/day)
   Function: antioxidant/flavonoid; histamine modulation.
   Mechanism: stabilizes mast cells; may reduce surface irritation.

7. N-acetylcysteine (NAC; 600–1,200 mg/day)
   Function: mucus/tear quality; antioxidant precursor (glutathione).
   Mechanism: thiol donor, reduces oxidative stress.

8. Lutein & Zeaxanthin (10 mg/2 mg day)
   Function: visual quality/retinal health; antioxidant support.

9. Coenzyme Q10 (100–200 mg/day)
   Function: mitochondrial antioxidant; supports cellular stress handling.

10. Magnesium (as tolerated, 200–400 mg/day)
    Function: stress modulation and neuromuscular relaxation (indirect).

11. Zinc (10–25 mg/day short courses if diet is poor)
    Function: cofactor in immune/repair enzymes.

12. β-Hydroxybutyrate (via dietary ketosis or exogenous salts/esters, clinician-guided)
    Function: theoretical NLRP3 inhibition (basic science).
    Mechanism: interferes with inflammasome activation. NCBI

13. Butyrate support via dietary fiber/fermented foods (no fixed dose; food-first)
    Function: gut-derived short-chain fatty acids that may damp NLRP3 signaling (preclinical).
    Mechanism: HDAC modulation/inflammasome effects. EyeWiki

14. Probiotics (evidence mixed)
    Function: general immune tone; may support ocular surface.

15. Oral hyaluronan (varies by product)
    Function: joint/ocular surface comfort adjunct; hydration support.

What to eat and what to avoid

1. Eat: fatty fish (salmon, sardines) 2–3×/week—omega-3s.

2. Eat: leafy greens, colored vegetables, berries—antioxidants.

3. Eat: nuts, seeds, olive oil—healthy fats.

4. Eat: high-fiber foods (oats, legumes, fermented foods)—butyrate support. EyeWiki

5. Drink: steady water intake; limit sugary drinks.

6. Limit: ultra-processed foods and high-sugar snacks (pro-inflammatory).

7. Limit: trans fats and excessive seed-oil deep-fried foods.

8. Limit: alcohol (can worsen sleep and inflammation).

9. Avoid: smoking/vape—surface irritation and oxidative stress.

10. Watch the salt: high-salt diets can worsen fluid balance in general; while corneal edema is local, a lighter hand with salt is sensible for overall health.

When to see a doctor (and red flags)

• At the very start of an attack (same day if possible): early anti-inflammatory drops may shorten the flare. EyeWiki

• If pain is severe, vision drops suddenly, or light hurts so much you can’t function.

• If you notice halos/colored rings with headache or nausea (rule out pressure spikes).

• If an attack lasts longer than a week or doesn’t improve after 48 hours of the usual plan.

• If attacks become more frequent or you feel vision is worse between attacks.

• If you use steroid drops: you’ll need eye-pressure checks. NCBI

FAQs

1) Is KEFH contagious?
No. It’s not an infection. It’s an inherited tendency for brief corneal inflammation.

2) Will I go blind?
Attacks are short-lived. Most people see well between episodes. A minority develop central haze after many years; surgery is rarely needed. NCBI

3) Why is it worse in cold wind or stress?
Cold and stress can nudge the over-reactive NLRP3 inflammasome in some people, triggering a flare. Not everyone has clear triggers. PubMed

4) Can children have KEFH?
Yes—often starts in childhood (median ~11 years). disorders.eyes.arizona.edu

5) Do I need genetic testing?
Not always. If the story is classic and there’s family history, your specialist may diagnose clinically. Testing helps in unclear or sporadic cases. EyeWiki

6) Is this the same as uveitis or herpetic endotheliitis?
No, but it can look similar, which is why KEFH is sometimes misdiagnosed at first. Experienced cornea specialists use history, exam, and occasionally lab tests to rule out mimics. ajo.com

7) What’s the main treatment during an attack?
A short course of steroid drops, sometimes with a cycloplegic, hypertonic saline, and oral pain relief. Your doctor will personalize the plan. EyeWikiNCBI

8) Are long-term steroids needed?
Usually no. Treatment is short and only during attacks. You’ll be monitored for eye pressure. NCBI

9) Can diet or supplements stop attacks?
There’s no proven supplement to prevent KEFH flares. A generally anti-inflammatory diet may support comfort and health. (See ideas above.)

10) Will this affect both eyes?
Most attacks are unilateral; some people experience alternating eyes or rarely both simultaneously. NCBI

11) Will it get better as I get older?
Yes—attacks often become less frequent and milder with age. disorders.eyes.arizona.edu

12) Can I wear contact lenses?
Usually yes between attacks if your cornea is healthy, but stop during flares. Your specialist will advise.

13) Is laser or transplant common?
No. Surgery is uncommon and reserved for persistent vision problems from scarring or chronic edema. NCBI

14) Is KEFH only in Finns?
It was first described and is most common in Finnish families, but cases exist in other European-ancestry groups. EyeWiki

15) What’s the biggest mistake to avoid?
Delaying evaluation during a first or atypical attack. Quick assessment helps exclude look-alike conditions and start treatment early. ajo.com

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 10, 2025.

PDF Document For This Disease Conditions References