Keratoendotheliitis fugax hereditaria (often shortened to KEFH) is a rare, inherited eye condition. People get short, repeat attacks where the clear window of the eye (cornea) becomes inflamed and water-logged (edematous). During an attack, one eye (sometimes both) gets red, painful, very light-sensitive, and vision gets blurry. These attacks usually start in childhood and tend to last 1–2 days (some sources say up to 2–5 days). Over many years, repeat attacks can leave faint scars in the center of the cornea that may slightly reduce vision. The disease is usually autosomal dominant, meaning one affected parent can pass it on. PubMedEye Disorders DatabaseNCBI
Scientifically, KEFH is linked to a single letter change in a gene called NLRP3 (this gene makes a protein nicknamed cryopyrin, part of the body’s inflammation “alarm system” called the NLRP3 inflammasome). The specific change is c.61G>C, which swaps one amino acid for another (p.Asp21His) and makes the inflammasome too easy to switch on, causing flares in the cornea. This variant has been confirmed in Finnish families and also appears at low frequency in other European populations. PubMedScienceDirect
During flares, microscope imaging shows tiny “pseudoguttata” spots on the cornea’s inner layer (the endothelium) and hazy swelling in the back layers of the cornea. Between attacks, the eye can look normal. PubMedEye Disorders Database
Keratoendotheliitis Fugax Hereditaria—often shortened to KEFH—is a rare, inherited eye condition that causes short, painful “attacks” of corneal inflammation. Affected people usually get unilateral (one-eye) episodes of redness, light sensitivity, blurry vision, and corneal swelling that last about 2–5 days, then settle. Over many years, repeated attacks can leave faint central corneal scars in some patients. KEFH is autosomal dominant (it tends to run in families), and modern research links it to a mutation in the NLRP3 gene (cryopyrin), which makes the eye’s innate immune system “too easy to trigger.” NCBIEyeWikiPubMed
KEFH is a genetic, “on-and-off” inflammation of the inner layer of the cornea (the endothelium) that flares for a few days and then calms down. During a flare, the clear window of the eye (the cornea) temporarily clouds and swells, so vision goes foggy and lights may look haloed. Pain, tearing, and redness are common. Between attacks, many people feel normal and see well. After years of attacks, some develop persistent central stromal opacities (light scars) that can mildly reduce vision. KEFH usually starts in childhood or early adulthood, often affects one eye at a time, and tends to improve in middle age (fewer, milder attacks). Triggers often include cold wind and stress. The driving cause is a hyper-reactive NLRP3 inflammasome (a molecular “alarm system” inside immune cells), which explains why the condition is grouped with cryopyrin-associated periodic syndromes (CAPS). EyeWikiScienceDirectPMCPubMed
Many people with KEFH are initially misdiagnosed (often as anterior uveitis/iritis), and it can take years to reach the correct diagnosis—so recognizing the pattern matters. PubMedFolkhälsan Research
Types
There aren’t official subtypes, but doctors often describe KEFH in useful patterns:
By laterality (which eye):
Unilateral (most common): one eye at a time.
Alternating bilateral: one eye, then the other on a different day.
Simultaneous bilateral (rare): both eyes together. EyeWiki
By age pattern:
Typical childhood onset (most begin ~age 6–12, median ~11).
Late onset (teen/adult first flare). Flares tend to ease with age. PubMedEye Disorders Database
By phase:
Acute attack: red, painful eye; corneal swelling; pseudoguttata; vision blur.
Between attacks: quiet eye, sometimes central corneal faint scars after years. PubMed+1
By disease burden:
Mild: few flares, no lasting haze.
Moderate: repeated flares with faint central haze.
Severe: many flares, central stromal opacities with mild chronic blur. PubMed
By genetics:
Familial (autosomal dominant): typical.
Sporadic (de novo): uncommon but reported; genetic testing can help confirm. EyeWiki
Causes
Root biological cause
NLRP3 gene mutation (c.61G>C; p.Asp21His): makes cryopyrin overly sensitive; inflammation switches on too easily. PubMedScienceDirect
Overactive NLRP3 inflammasome: lowers the threshold to start an eye-inflammation attack. Helda
Autosomal-dominant inheritance: one changed copy from a parent is enough to cause the condition. Eye Disorders Database
Founder effect in Northern Europe: variant enriched in Finnish/nearby ancestry (still rare overall). PubMed
Occasional de novo mutation: new mutation in a child even if parents aren’t affected. EyeWiki
Commonly reported attack triggers (not the cause of the disease, but things that may set off a flare)
Psychological stress (or shifts in stress). PubMed
Lack of sleep/fatigue (reported). Wiley Online Library
Mild viral illness (reported anecdotally). EyeWiki
Ocular micro-trauma or irritation (e.g., rubbing, environmental grit) may be associated in some reports. ScienceDirect
General systemic immune “priming” (body is more inflamed than usual) can lower the flare threshold. (Inference from NLRP3 biology and CAPS family.) PMC
Less frequent or possible contributors noted in series/reviews
Air drafts/dry air (overlaps with “cold air”). Helda
Recent minor eye infection (diagnostic confusion with infectious keratitis is common; infections per se are not KEFH, but intercurrent infections may confuse/trigger care). Mayo Clinic
Misdiagnosed uveitis episodes can delay correct care and allow more attacks (not a cause, but a contributor to burden). PubMed
Allergy-like histamine state (antihistamines sometimes help symptoms; evidence mainly symptomatic). EyeWiki
Windy outdoor work/exposure (environmental cold-wind overlap). PubMed
Eye surgery/trauma history (rarely discussed; included as potential nonspecific trigger for corneal inflammation in general). ScienceDirect
Family history (isn’t a trigger but predicts risk strongly). Eye Disorders Database
Age (attacks more frequent/severe in youth; tend to soften with age). PubMed
Unknown factors (many attacks happen without a clear trigger). PubMed
Important note: The gene mutation is the cause; items 6–20 are reported triggers or contributors that may bring on a flare in someone who already has KEFH.
Symptoms
Eye pain (often moderate to severe). NCBI
Red eye (conjunctival injection). NCBI
Blurred vision (from corneal swelling/haze). EyeWiki
Light sensitivity (photophobia)—bright light hurts. Eye Disorders Database
Watering/tearing (lacrimation). NCBI
Gritty/foreign-body feeling. NCBI
Colored halos around lights (when the cornea is swollen). NCBI
Double vision (diplopia) at times (from blur/halos, not from eye muscles). NCBI
Mild headache or “stiff neck” feeling (described in some histories). EyeWiki
One-sided nasal stuffiness/runny nose on the same side as the affected eye (reported). EyeWiki
Eye becomes very tender to touch (pain worsens with palpation). EyeWiki
Temporary drop in vision acuity during attacks. PubMed
Mild anterior chamber reaction (tiny inflammatory cells seen by the doctor’s microscope; patients feel it as pain/photophobia). PubMed
Between attacks: usually no symptoms, but central corneal haze can leave mild “permanent” blur in older patients with many flares. PubMed
Attacks recur (often several times per year), then taper with age. PubMed
Diagnostic tests
A) Physical exam (done in the clinic chair)
History pattern check
The doctor asks about short, repeat flares starting in childhood with pain/redness/blur in one eye that settle within a few days. This pattern strongly suggests KEFH and helps separate it from infections or classic uveitis. PubMed+1Visual acuity (eye chart)
Measures how much the attack reduces vision and tracks recovery between flares. (Usually returns to baseline unless scarring accumulates.) PubMedPenlight photophobia test
Bright light hurts because the inflamed cornea is sensitive—this is a simple, quick check consistent with corneal disease. Eye Disorders DatabaseExternal exam
Looks for redness, tearing, lid squeezing—the “red painful photophobic eye” pattern that points to the cornea. NCBIPupil exam
Ensures there’s no serious nerve problem; also helps rule out other causes of visual loss when the cornea clears. (Usually normal in KEFH.) Eye Disorders Database
B) “Manual” office tests (hands-on or simple tools)
Slit-lamp biomicroscopy during a flare
The key exam: shows corneal edema/haze and pseudoguttata (tiny dark “holes” between endothelial cells), sometimes a mild anterior chamber reaction. Between flares, the eye may look normal or show central stromal opacities from prior attacks. PubMed+1Fluorescein dye + blue light
Helps confirm the cornea is the source of symptoms and rules out epithelial ulcers that suggest infections (bacterial, herpes, etc.). Mayo ClinicIntraocular pressure (IOP) check
Usually normal in KEFH but measured to exclude angle-closure glaucoma or steroid-response pressure rise if drops are used. EyeWikiCotton-wisp corneal sensitivity
Normal sensitivity supports non-herpetic inflammation; reduced sensitivity leans to herpetic keratitis—a key differential. Mayo ClinicSchirmer tear test / tear breakup time
Not diagnostic for KEFH itself, but helps rule out dry eye as a cause of irritation or muddled symptoms. (Supportive test.) Mayo Clinic
C) Lab & pathological tests
Genetic testing for NLRP3 (targeted variant c.61G>C; p.Asp21His)
The confirmatory test when the story fits (especially with family history or atypical findings). It proves the diagnosis in uncertain cases. PubMedInflammatory blood markers (CRP/ESR) during a flare
Often normal in KEFH (eye-limited), but checking can exclude systemic inflammatory diseases; in CAPS relatives, these can rise, so clinicians sometimes look. PMCAqueous tap PCR (rare/only if needed)
If a first attack looks unusual, doctors may test eye fluid to exclude herpes endotheliitis before using strong steroids. (This is uncommon but cautious medicine.) Mayo ClinicHistopathology (very rare; after corneal transplant if ever done)
Pathology from a corneal “button” has shown inflammatory cell changes consistent with prior flares. This is not routine—only reported in research/exceptional cases. PubMed
D) Electrodiagnostic tests (seldom needed; used to rule out other causes of blurred vision)
Visual evoked potential (VEP)
Not for diagnosis of KEFH; occasionally used if vision remains poor after the cornea clears to confirm the problem isn’t in the optic nerve/brain. (Rare scenario.)
(Included here because you asked for electrodiagnostic items; most KEFH patients never need this.)Electroretinogram (ERG)
Similarly, rarely used. It checks retinal function if clinicians suspect another condition hiding behind the corneal symptoms.
(Again, rarely indicated in typical KEFH.)
E) Imaging tests (very helpful in KEFH)
Specular microscopy
Photographs the corneal endothelium. During a flare it shows pseudoguttata (dark, non-reflective gaps); even between attacks it may show mild cell shape/size changes. This is one of the most informative tests for KEFH. EyeWikiIn vivo confocal microscopy (IVCM)
A higher-resolution “live” microscope that has shown hyper-reflective inflammatory cells in the corneal stroma/endothelium during attacks and helps confirm the characteristic pattern. PubMedCorneal pachymetry (ultrasound or optical)
Measures thickness, which increases by ~5–14% during a flare and returns to baseline afterward. EyeWikiAnterior segment OCT (AS-OCT)
Non-contact scan that documents posterior stromal haze/edema and helps track resolution after the attack. Corneal topography may also be used to document changes or rule out other corneal conditions. PubMed
Non-pharmacological treatments
Important: These measures support comfort, safety, and trigger management. They do not replace medical therapy when a flare is active.
Cold-avoidance plan
Purpose: Reduce flares thought to be triggered by cold wind or air.
Mechanism: Avoids a physical trigger that may perturb corneal homeostasis and NLRP3 activation. Practical tips: wrap-around glasses outdoors; avoid direct AC venting; use a scarf mask in cold weather. ScienceDirectStress-reduction routine
Purpose: Lower stress-linked flares.
Mechanism: Calms autonomic and inflammatory pathways that may prime NLRP3 activation. Examples: paced breathing, short daily mindfulness, light exercise if your ophthalmologist okays it. PMCMoisture-chamber eyewear
Purpose: Shield the ocular surface from dry, windy environments.
Mechanism: Reduces evaporation and wind shear that can irritate the corneal surface during recovery.Humidification of rooms
Purpose: Keep ambient humidity 40–50% to reduce surface dryness and irritation.Screen-time hygiene
Purpose: Minimize light sensitivity and strain.
Mechanism: Frequent blink breaks (20-20-20), reduce glare, use night modes during recovery.Sunglasses/photophobia control
Purpose: Comfort in bright light.
Mechanism: Filters incoming light; polarized lenses help with halos.Driving/operating machinery pause during a flare
Purpose: Safety.
Mechanism: Temporary blurred vision + halos increases risk.Sleep optimization
Purpose: Recover faster; lower inflammatory tone.
Mechanism: Sleep supports immune regulation and healing.Trigger diary
Purpose: Identify personal precipitating factors (cold, stress bursts, long outdoor exposure).
Mechanism: Pattern recognition → tailored avoidance.Eye protection for dusty tasks
Purpose: Reduce micro-trauma/irritation.
Mechanism: Shields cornea from particulate triggers (gardening, sanding).Contact lens holiday during/after attacks
Purpose: Prevent extra stress on the cornea while it recovers.
Mechanism: Allows endothelial pump and surface to normalize.Cool compresses (brief, clean)
Purpose: Comfort for pain/photophobia.
Mechanism: Transient vasoconstriction → symptom relief. (Avoid pressure on the globe.)Hydration & blink-lubrication habits
Purpose: Support surface comfort.
Mechanism: Adequate hydration and conscientious blinking reduce surface dryness that can worsen light sensitivity (supportive only).Workplace adjustments
Purpose: Reduce exposure (e.g., cold warehouse, direct fan/AC).
Mechanism: Environmental control.Protective eyewear in sports
Purpose: Reduce accidental corneal impacts (micro-trauma).Family education
Purpose: Because KEFH is autosomal dominant, relatives may benefit from awareness and prompt evaluation during first attacks. Eye Disorders DatabaseEarly-action plan
Purpose: Have pre-agreed steps with your eye doctor for the start of a flare (e.g., same-day visit, what to do if clinic is closed).
Mechanism: Shortens time to appropriate care.Avoid unnecessary topical irritants
Purpose: Don’t self-medicate with decongestant “get-the-red-out” drops.
Mechanism: Rebound redness and surface dryness can worsen comfort.Eye-rubbing avoidance
Purpose: Rubbing can aggravate symptoms and surface trauma.General anti-inflammatory lifestyle
Purpose: Lower background inflammatory load.
Mechanism: Regular physical activity (as cleared by your physician), Mediterranean-style diet pattern, and smoking cessation all support systemic immune balance.
Drug treatments
There is no single, proven “curative” drug for KEFH. Management is based on case reports/series and expert opinion; therapy targets symptom relief during attacks and, in select severe/recurrent cases, upstream IL-1/NLRP3 pathways extrapolated from CAPS therapy. Always follow your ophthalmologist’s dosing and taper. EyeWiki
1) Topical corticosteroids (e.g., prednisolone acetate 1%)
Class: Ophthalmic steroid anti-inflammatory.
Dose/time (typical): 1 drop 4×/day during an acute attack for 3–5 days, then taper over several days per clinician direction.
Purpose: Reduce intra-corneal inflammation and endothelial irritation to shorten symptoms.
Mechanism: Broad suppression of cytokine signaling.
Side effects: Elevated intraocular pressure (IOP), delayed healing, cataract with prolonged/repeated use; infection risk if misused. EyeWiki
2) Soft-steroid alternative (e.g., loteprednol 0.5%)
Class: Ester-based topical steroid.
Use: Similar goals as prednisolone; may be chosen for a lower IOP-rise tendency.
Note: Still requires monitoring.
3) Topical NSAID (e.g., ketorolac 0.5%)
Class: Non-steroidal anti-inflammatory.
Dose/time (typical): 1 drop 3–4×/day for a few days in an attack.
Purpose: Pain/light sensitivity relief.
Mechanism: COX inhibition → lower prostaglandins.
Side effects: Stinging on instillation; with prolonged use, rare corneal adverse effects—so keep short and supervised. EyeWiki
4) Oral NSAID (e.g., ibuprofen 400 mg)
Class: Systemic analgesic/anti-inflammatory.
Dose/time (typical adult): 400 mg every 6–8 h with food, short course.
Purpose: Pain control; may help photophobia.
Cautions: Avoid in peptic ulcer disease, kidney disease, or with anticoagulants unless cleared.
5) Oral antihistamine (e.g., cetirizine 10 mg daily)
Class: H1 blocker.
Purpose: May help comfort (sedation can reduce photophobia/tearing) per reports.
Mechanism: Sedative and mild anti-itch effects; does not treat the genetic cause. EyeWiki
6) Hypertonic saline 5% (drops/ointment)
Class: De-edema agent (topical saline).
Dose/time: Drops 4–6×/day and/or ointment at bedtime during recovery.
Purpose: Draws fluid out of the cornea to clear haze faster (supportive).
Cautions: Stinging; not all patients find it helpful.
7) Cycloplegic drops (e.g., cyclopentolate 1%)—select cases
Class: Antimuscarinic.
Dose/time: 1 drop 2–3×/day briefly if ciliary spasm–type pain is prominent.
Purpose: Light sensitivity and ache relief.
Cautions: Blurs near vision; avoid driving after instillation.
8) Lubricating drops/gel (preservative-free preferred during flares)
Class: Ocular surface protectants.
Use: QID or more for comfort; particularly helpful when photophobia is high.
Note: Supportive only.
9) IL-1 receptor antagonist (anakinra) — off-label, specialist use
Class: Targeted anti-inflammatory (biologic).
Dose/time (typical CAPS): 100 mg subcutaneous daily (weight-based in children).
Purpose: In rare, severe/refractory KEFH linked to NLRP3 hyperactivity—especially if there are systemic CAPS-like signs—some clinicians extrapolate from CAPS evidence.
Mechanism: Blocks IL-1 signaling downstream of NLRP3.
Side effects: Injection-site reactions, infection risk; needs systemic specialist oversight. Evidence in KEFH is limited (case-level). PMCScienceDirect
10) IL-1β monoclonal antibody (canakinumab) — off-label, specialist use
Class: Targeted biologic.
Dose/time (common CAPS adult): 150 mg SC every 8 weeks (weight-based variants).
Purpose: Similar rationale as anakinra; not standard for KEFH but biologically plausible in selected severe contexts.
Side effects: Infection risk; cost; requires rheumatology/ophthalmology co-management. Frontiers
What’s not recommended by default: Empiric antivirals or antibiotics unless your clinician suspects an infectious mimic (e.g., HSV endotheliitis). KEFH itself is not an infection.
Dietary, molecular & supportive supplements
Key disclaimer: None of these has direct clinical evidence for KEFH. Think of them as adjuncts for general anti-inflammatory health. Discuss all supplements with your clinician, especially if pregnant, on blood thinners, or with kidney/liver disease.
Omega-3 fatty acids (fish oil) — 1–2 g/day EPA+DHA.
Function/mechanism: Pro-resolving mediators that generally dampen inflammatory signaling.Vitamin D3 — check level; typical 800–2000 IU/day if low.
Mechanism: Immune modulation; correct deficiency.Vitamin C — 200–500 mg/day.
Mechanism: Antioxidant support for ocular surface health.Magnesium — 200–400 mg/day (glycinate/citrate).
Mechanism: Neuromuscular relaxation; may help stress responses.Curcumin (with piperine or as a bioavailable formulation) — 500–1000 mg/day.
Mechanism: NF-κB and inflammasome-modulating properties in preclinical studies.Quercetin — 250–500 mg/day.
Mechanism: Flavonoid with antioxidant/anti-inflammatory effects.Resveratrol — 100–200 mg/day.
Mechanism: Sirtuin-linked anti-inflammatory signaling.N-acetylcysteine (NAC) — 600 mg 1–2×/day.
Mechanism: Glutathione precursor; antioxidant.Lutein + Zeaxanthin — per label (often 10 mg/2 mg daily).
Mechanism: Ocular antioxidants; general retinal health.Coenzyme Q10 — 100–200 mg/day with fat.
Mechanism: Mitochondrial antioxidant.Green tea extract (EGCG) — standardized dose per label.
Mechanism: Polyphenol anti-inflammatory properties.Probiotics — daily, multi-strain.
Mechanism: Gut-immune crosstalk; foundational support.Zinc — 10–25 mg/day (short term unless deficient).
Mechanism: Immune enzyme cofactor; avoid excess.Saffron extract — per label.
Mechanism: Antioxidant; small ocular studies in other contexts.Melatonin (evening, 0.5–3 mg)
Mechanism: Sleep regulation; sleep supports immune balance.
Regenerative / stem-cell” drugs
Reality check: For KEFH, there is no approved “immune reset” or regenerative therapy. Because KEFH stems from NLRP3/IL-1 overactivation, the most biologically targeted choices—anakinra and canakinumab—come from CAPS care and remain off-label for KEFH (see Drug #9–10 above). Stem-cell or endothelial-cell therapies are being developed for other corneal endothelial diseases (like bullous keratopathy or Fuchs), and show promise, but they’re not designed for KEFH, where the endothelium is mostly normal between attacks. If the eye develops fixed scarring, surgery—not cell therapy—may be considered. New England Journal of MedicineScienceDirectPMC
A candid list of what clinicians may discuss in complex cases (specialist only):
Anakinra (IL-1 receptor antagonist) — see Drug #9 above.
Canakinumab (anti-IL-1β) — see Drug #10 above.
Other NLRP3-pathway ideas (research stage) — small-molecule NLRP3 inhibitors are being studied outside ophthalmology; no KEFH trials yet. PMC
Cultured human corneal endothelial cell (CEC) injection — viable for endothelial failure (not KEFH); early trials/long-term cohorts show safety/efficacy in other diseases. New England Journal of MedicineScienceDirect
ROCK-inhibitor–assisted CEC therapy — investigational for endothelial dysfunction; not a KEFH treatment. Lippincott Journals
iPSC-derived CECs — first-in-human studies are emerging for endothelial disease, not KEFH. Cell
Surgeries
Most KEFH patients never need surgery. Procedures are reserved for rare cases with persistent, visually significant central stromal scarring after years of attacks.
Phototherapeutic keratectomy (PTK)
What it is: An excimer laser gently polishes the front corneal layers to reduce superficial scars/irregularities.
Why: If the opacity is superficial and disrupts vision/glare.
Notes: Recurrence of scars is possible over time; careful selection is vital. EyeWikiDeep anterior lamellar keratoplasty (DALK)
What: Surgeons replace the corneal stroma while preserving Descemet’s membrane and endothelium.
Why: Deeper stromal scarring without endothelial failure.
Notes: Lower rejection risk than full-thickness grafts.Penetrating keratoplasty (PK)
What: Full-thickness corneal transplant.
Why: Extensive scarring when other options aren’t suitable.
Notes: Highest rejection/astigmatism risk; reserved for selected cases.Descemet membrane endothelial keratoplasty (DMEK)
What: Replaces the endothelium.
Why: Generally not a KEFH operation (the endothelium is usually fine between attacks); considered only if independent endothelial failure occurs from another cause.Photorefractive “surface smoothing” adjuncts
What: Customized laser smoothing for glare/irregularity in select shallow scars, sometimes after PTK assessment.
Why: Improve optics when opacity is very superficial. PubMed
Prevention strategies
Protect from cold wind (wrap-around glasses, scarf). ScienceDirect
Manage stress (daily micro-practices). PMC
Set an early-flare plan with your ophthalmologist (same-day access).
Avoid eye rubbing.
Use moisture-chamber eyewear in harsh environments.
Humidify indoor air.
Pause contact lenses during/after flares.
Keep protective eyewear for dusty/impact-risk tasks.
Educate family members (autosomal dominant). Eye Disorders Database
Maintain general health (sleep, activity, balanced diet) to support immune stability.
When to see a doctor
Call your eye doctor promptly if you have any of the following:
A new attack (especially if it’s your first or worse than usual).
Severe pain, marked light sensitivity, or rapidly worsening blur.
Symptoms not improving within 2–3 days, or blur persisting beyond 2–3 weeks.
Both eyes affected at once, or attacks >3–4 times/year.
Systemic symptoms suggestive of CAPS (fevers, rash, joint pains) — you may need genetics/rheumatology input. NCBI
What to eat and what to avoid
What to favor
Mediterranean-style pattern: vegetables, fruits, legumes, whole grains, nuts, olive oil, fish (omega-3s).
Hydration: regular water intake supports surface comfort.
Protein: helps tissue repair (fish, poultry, beans, dairy if tolerated).
Micronutrients: foods rich in vitamin C (citrus, peppers), vitamin D (fortified dairy/fish), and lutein/zeaxanthin (leafy greens).
What to limit
Ultra-processed, high-sugar foods that can amplify systemic inflammation.
Very salty meals that may worsen temporary fluid retention.
Excess alcohol, which can disturb sleep and immunity.
Any food you personally notice precedes flares (use your trigger diary).
Frequently asked questions (FAQs)
1) Is KEFH contagious?
No. It’s genetic and inflammatory, not infectious.
2) Will every family member get it?
KEFH is autosomal dominant, so each child of an affected parent has about a 50% chance to inherit the variant—but actual expression varies. Genetic counseling can help. Eye Disorders Database
3) How long do attacks last?
Most flares resolve in 2–5 days; vision blur may linger weeks as the cornea clears. PubMed
4) What starts an attack?
Often cold wind or stress, but triggers differ; some attacks occur without any obvious reason. Keep a diary. ScienceDirect
5) Can it cause permanent damage?
Many patients recover fully between attacks; however, repeated flares can leave central stromal opacities in some people. NCBI
6) Are steroids safe for every attack?
Short courses can help, but they must be supervised due to IOP rise and other risks. Never self-start steroids without guidance. EyeWiki
7) Do antivirals help?
Not for KEFH itself. Antivirals are used only if your doctor suspects herpetic endotheliitis, a different condition.
8) Are biologics (anakinra/canakinumab) a cure?
No cure. In rare, severe cases with systemic CAPS features, specialists may consider them off-label; evidence in KEFH is limited. ScienceDirect
9) Can supplements stop attacks?
No supplement is proven to prevent KEFH flares. Some may support general anti-inflammatory health, but they don’t replace medical care.
10) Will I need surgery?
Unlikely. Surgery is only for persistent, vision-limiting scars, and the choice depends on scar depth (PTK vs. lamellar vs. PK). EyeWiki
11) Can I wear contact lenses?
Yes, between attacks if your doctor approves. Avoid contacts during flares and early recovery.
12) Is KEFH only in Finland?
It was first recognized in Finland, but NLRP3-linked KEFH has also been identified in non-Finnish Europeans. PubMed
13) Why was I told it’s “uveitis” or “HSV” before?
KEFH can mimic these conditions. Diagnostic delays/mislabels are reported, which is why history + exam + (sometimes) genetics matter. Ajo
14) Can children have KEFH?
Yes—childhood onset is common; families should have a plan for prompt evaluation in early attacks. EyeWiki
15) What’s the outlook?
Generally good: attacks are self-limited and often lessen with age. Long-term scarring can occur in some; staying on top of trigger control and early treatment helps. Ajo
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 09, 2025.
Keratoconus
