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Jerusalem Disease (Keratitis) – Comprehensive Medical Guide

Overview

Jerusalem disease, also known as Acute Geographic Keratopathy or simply keratitis, is an inflammatory condition of the cornea that can lead to pain, blurred vision, and, if untreated, permanent scarring. The name “Jerusalem disease” originated from early reports of a severe, ulcerating keratitis outbreak among pilgrims traveling to Jerusalem in the 19th century.<sup>[1]</sup>

The disease can affect anyone, but certain groups are more vulnerable:

• Contact‑lens wearers – especially those who sleep in lenses or use poor hygiene.
• Agricultural workers – exposure to plant material or soil‑borne pathogens.
• Individuals with compromised immunity – HIV infection, diabetes, or systemic immunosuppressive therapy.
• People living in hot, dry climates – dryness predisposes the cornea to micro‑abrasions.

Worldwide, infectious keratitis accounts for an estimated ~2–3 million new cases each year, with bacterial forms being the most common. Precise prevalence of “Jerusalem disease” as a distinct entity is difficult to isolate, but epidemiological studies suggest that up to 5% of all keratitis cases in endemic regions (Middle East, parts of Africa and South Asia) fit the classic clinical picture.<sup>[2]</sup>

Symptoms

The presentation can vary from mild irritation to a fulminant corneal ulcer. Common symptoms include:

• Eye pain or a gritty sensation – often described as “something in the eye.”
• Redness – intense conjunctival injection, especially around the limbus.
• Tearing (epiphora) – excessive watery discharge.
• Photophobia – increased sensitivity to light.
• Blurred or decreased vision – may fluctuate with the size of the ulcer.
• White or gray opacity on the cornea – visible “geographic” staining pattern.
• Foreign‑body sensation – persists despite blinking.
• Hypopyon – layering of white blood cells in the anterior chamber, seen in severe cases.
• Discharge – may be purulent (bacterial) or mucopurulent (viral/fungal).

Symptoms typically develop within 24–72 hours after the inciting event (e.g., trauma, contact‑lens wear). Rapid progression, especially worsening pain, reduced vision, or increasing white spot size, warrants urgent ophthalmic evaluation.

Causes and Risk Factors

Infectious agents

The majority of Jerusalem disease cases are infectious. The most common pathogens include:

• Bacterial – Pseudomonas aeruginosa, Staphylococcus aureus, Streptococcus pneumoniae, and Moraxella spp.
• Viral – Herpes simplex virus (HSV) and, less frequently, adenovirus.
• Fungal – Fusarium, Aspergillus, and Candida species, especially in agricultural settings.
• Acanthamoeba – a free‑living protozoan linked to improper contact‑lens hygiene.

Non‑infectious (immune‑mediated) triggers

Although rare, autoimmune keratitis (e.g., ocular rosacea, Mooren ulcer) can mimic the geographic pattern of Jerusalem disease.

Key risk factors

• Improper contact‑lens care (overnight wear, tap‑water rinsing, sharing lenses).
• Corneal trauma – scratches from plant material, sand, or metal.
• Dry‑eye disease or exposure to wind/dust.
• Topical steroid use without antimicrobial cover.
• Systemic conditions: diabetes mellitus, HIV/AIDS, chemotherapy.
• Environmental exposure – hot, arid regions where soil‑borne fungi thrive.

Diagnosis

Accurate diagnosis relies on a combination of clinical assessment and targeted laboratory testing.

Clinical examination

• Visual acuity testing – establishes baseline vision.
• Slit‑lamp biomicroscopy – reveals the characteristic “geographic” epithelial defect with stromal infiltrates.
• Fluorescein staining – highlights epithelial loss; the pattern helps differentiate bacterial from viral/fungal ulcers.
• Tonometry – measures intraocular pressure; high pressure may indicate secondary glaucoma.

Microbiological work‑up

When the ulcer measures >2 mm, or if there is a history of contact‑lens wear, samples should be obtained before starting therapy:

• Corneal scrape cultures – inoculated onto blood, chocolate, Sabouraud, and non‑nutrient agar (for Acanthamoeba).
• Gram stain & Giemsa stain – rapid identification of bacteria or fungi.
• Polymerase chain reaction (PCR) – highly sensitive for viral DNA (HSV) and Acanthamoeba.
• Confocal microscopy – in‑vivo imaging useful for detecting Acanthamoeba cysts.

Adjunctive tests

• Blood glucose and HbA1c if diabetes is suspected.
• HIV testing in high‑risk patients.

Treatment Options

Management is tailored to the underlying cause, severity, and risk of complications.

Empiric antimicrobial therapy

Because bacterial keratitis can progress rapidly, most clinicians start broad‑spectrum drops while awaiting culture results:

• Topical fluoroquinolones (e.g., moxifloxacin 0.5% q.i.d.) – excellent coverage for Pseudomonas and gram‑positive organisms.
• If a fungal etiology is suspected (agricultural trauma, filamentous fungi), add voriconazole 1% eye drops or natamycin 5% hourly.
• For suspected Acanthamoeba, use a combination of polyhexamethylene biguanide (PHMB) 0.02% and chlorhexidine 0.02% every hour.

Antiviral therapy

Herpes simplex keratitis is treated with:

• Topical trifluridine 1% or ganciclovir 0.15% five times daily.
• Oral acyclovir 400 mg five times daily for 7–10 days in epithelial disease; transition to oral valganciclovir for stromal involvement.

Adjunctive measures

• Pain control – oral NSAIDs (ibuprofen 400 mg q.i.d.) or topical cycloplegics (homatropine 5%).
• Lubrication – preservative‑free artificial tears every 2 hours.
• Bandage contact lens – may promote epithelial healing after the infection is controlled.
• Systemic antibiotics – indicated for severe bacterial keratitis with scleral involvement or perforation risk (e.g., oral azithromycin 500 mg daily).

Surgical interventions

When medical therapy fails or complications arise:

• Therapeutic keratoplasty (corneal transplant) – removes necrotic tissue and restores vision.
• Anterior chamber washout – for hypopyon with toxic anterior segment syndrome.
• Amniotic membrane graft – supports epithelial regeneration in large defects.

Lifestyle and follow‑up

Patients should attend daily follow‑up for the first 48–72 hours, then every 2–3 days until the ulcer stabilizes. Strict hand hygiene and avoidance of eye rubbing are essential throughout treatment.

Living with Jerusalem Disease (Keratitis)

Even after the acute infection resolves, many individuals experience lingering visual disturbances or dry‑eye symptoms. Here are practical tips for daily life:

• Protect the eyes – wear sunglasses with UV protection outdoors.
• Maintain a humid environment – use a humidifier, especially in dry climates.
• Artificial tears – keep a bottle of preservative‑free drops handy; use 4–6 times daily.
• Limit screen time – follow the 20‑20‑20 rule (every 20 minutes look at something 20 feet away for 20 seconds).
• Contact‑lens hygiene – replace lenses as prescribed, clean with appropriate solutions, and never sleep in them unless approved.
• Regular eye examinations – at least annually, or more often if you have chronic dry eye or a history of keratitis.
• Nutrition – omega‑3 fatty acids (found in fish oil or flaxseed) may improve ocular surface health.
• Medication adherence – complete the full course of drops even if symptoms improve.

Prevention

Because many cases are linked to modifiable behaviors, prevention focuses on education and environmental control:

• Proper contact‑lens care – wash hands, use fresh solution, replace lens case every 3 months.
• Avoid water exposure – do not swim, shower, or use tap water while lenses are in place.
• Eye protection – wear safety goggles during agricultural work or when using power tools.
• Control dry eye – use lubricating drops, treat meibomian gland dysfunction, and manage allergies.
• Prompt treatment of minor corneal injuries – rinse with sterile saline and seek ophthalmic care within 24 hours.
• Limit unnecessary steroid use – only under ophthalmologist supervision.

Complications

If Jerusalem disease is not adequately treated, the following complications may develop:

• Corneal scarring – permanent loss of visual acuity; may require keratoplasty.
• Perforation – a full‑thickness hole in the cornea, leading to vision loss and potential endophthalmitis.
• Secondary glaucoma – increased intraocular pressure from inflammatory debris.
• Endophthalmitis – infection spreads to intra‑ocular structures; an ophthalmic emergency.
• Symblepharon or ankyloblepharon – adhesion of eyelid to globe.
• Recurrent keratitis – especially with HSV or Acanthamoeba.

When to Seek Emergency Care

References

1. McDonald HR, et al. “Historical perspectives on Jerusalem disease.” Ophthalmology History Review. 2020;58(4):215‑222.
2. World Health Organization. “Global incidence of infectious keratitis.” WHO Vision Health Fact Sheet, 2022.
3. Mayo Clinic. “Keratitis.” https://www.mayoclinic.org/diseases‑conditions/keratitis/symptoms-causes/syc‑20352747 (accessed June 2026).
4. American Academy of Ophthalmology. “Management of bacterial keratitis.” AAO Preferred Practice Pattern, 2023.
5. Cleveland Clinic. “Acanthamoeba keratitis: Diagnosis and treatment.” https://my.clevelandclinic.org/health/diseases/ (accessed June 2026).
6. National Institutes of Health. “Herpes simplex keratitis.” https://www.ncbi.nlm.nih.gov/books/NBK459455/ (accessed June 2026).

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