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Keratitis Sicca Secondary to Sjögren’s Syndrome

Overview

Keratitis sicca (dry‑eye keratitis) is inflammation of the cornea that results from severe tear‑film deficiency. When it occurs in the setting of Sjӧgren’s syndrome, an autoimmune disease that attacks the body’s moisture‑producing glands, the condition is termed “keratitis sicca secondary to Sjögren’s syndrome.”

• Who it affects: Primarily adult women (≈90 % of Sjögren’s cases) aged 40–60, although men and younger adults can be affected.
• Prevalence: Sjögren’s syndrome affects about 0.1–0.4 % of the U.S. population (≈300,000–1.2 million people). Up to 70 % of these patients develop clinically significant dry‑eye disease, and a subset progresses to keratitis sicca.

Because the cornea is the eye’s front window, chronic dryness can damage its surface, leading to pain, visual blur, and, if untreated, permanent vision loss.

Symptoms

Symptoms often develop gradually and may be mistaken for ordinary eye irritation. Below is a comprehensive list with brief explanations.

• Persistent gritty or sandy sensation – the classic “foreign‑body” feeling.
• Burning or stinging pain – worsens in dry, windy, or air‑conditioned environments.
• Redness (conjunctival injection) – due to surface inflammation.
• Excessive tearing (reflex tearing) – paradoxical response when eyes attempt to compensate for dryness.
• Blurred vision that improves with blinking – the tear film is unstable.
• Photophobia – sensitivity to light.
• Eye fatigue or heaviness – especially after reading or screen use.
• Difficulty wearing contact lenses – lenses exacerbate dryness.
• Visible specks or mucus on the eye surface – from damaged epithelial cells.
• Feeling that the eye is “closed” or “shut” even when open.

Causes and Risk Factors

Underlying Mechanism

Sjögren’s syndrome is a systemic autoimmune disorder in which lymphocytic infiltration destroys exocrine glands, notably the lacrimal (tear) and salivary glands. The resulting tear‑film deficit leads to osmotic stress, epithelial cell loss, and inflammation of the cornea – the hallmark of keratitis sicca.

Risk Factors

• Female sex – hormonal influences appear to increase autoimmunity.
• Age >40 years – glandular degeneration accumulates over time.
• Existing primary or secondary Sjögren’s syndrome – the primary cause.
• Other autoimmune diseases – rheumatoid arthritis, systemic lupus erythematosus, or thyroid disease raise risk.
• Medications that reduce tear production – antihistamines, antidepressants, β‑blockers, isotretinoin.
• Environmental exposures – low humidity, wind, air‑conditioned rooms, and prolonged screen time.
• History of ocular surgery or trauma – can impair tear‑film stability.

Diagnosis

Diagnosing keratitis sicca secondary to Sjögren’s syndrome requires a combination of patient history, clinical examination, and specific tests to assess tear production and corneal health.

Step‑by‑step diagnostic approach

1. Medical history – documentation of Sjögren’s diagnosis, medication list, environmental exposures, and symptom chronology.
2. Visual acuity test – to establish any baseline vision loss.
3. Slit‑lamp examination – the ophthalmologist looks for corneal punctate epithelial erosions, fluorescein staining patterns, and conjunctival changes.
4. Tear‑film tests:
   • Schirmer I test – filter paper placed under the lower eyelid for 5 minutes; <5 mm wetting suggests severe dry eye.
   • Tear break‑up time (TBUT) – fluorescein dye is used; a TBUT <10 seconds indicates instability.
5. Ocular surface staining – fluorescein, lissamine green, or rose bengal highlight damaged epithelial cells.
6. Meibomian gland evaluation – to rule out concurrent evaporative dry eye.
7. Laboratory confirmation of Sjögren’s (if not already established) – anti‑SSA/Ro and anti‑SSB/La antibodies, ANA, rheumatoid factor; salivary gland biopsy may be used.

Diagnostic criteria from the American College of Rheumatology/European League Against Rheumatism (ACR/EULAR) are often cited for Sjögren’s, while the Dry Eye Workshop (DEWS) classification guides ocular assessment.<sup>[1]</sup>

Treatment Options

Treatment is multimodal, aiming to restore tear‑film volume, reduce inflammation, protect the cornea, and address systemic disease.

Topical Therapies

• Artificial tears – preservative‑free formulations (e.g., sodium hyaluronate, carboxymethylcellulose) used 4–6 times daily.
• Lubricating ointments or gels – applied at night for prolonged surface coverage.
• Cycloplegic agents (e.g., 0.5 % cyclopentolate) – reduce pain from corneal nerve irritation.
• Anti‑inflammatory drops:
  • Ciclosporin A 0.05 % (Restasis) – improves tear production by modulating T‑cell activity.
  • Lifitegrast 5 % (Xiidra) – inhibits lymphocyte function‑associated antigen‑1 (LFA‑1) and reduces inflammation.
• Short‑course topical corticosteroids – prednisolone acetate 1 % for acute flares, usually tapered over 2–4 weeks.

Systemic Therapies (for underlying Sjögren’s)

• Hydroxychloroquine – disease‑modifying antirheumatic drug (DMARD) that can improve systemic and ocular symptoms.
• Systemic immunosuppressants (e.g., methotrexate, azathioprine) – reserved for severe extra‑glandular disease.
• Biologic agents – rituximab (anti‑CD20) has shown benefit in refractory dry‑eye disease in randomized trials.<sup>[2]</sup>

Procedural Interventions

• Punctal plugs – silicone or collagen plugs inserted into the lacrimal puncta to retain tears; effective in ~70 % of patients.
• Tear‑film “rejuvenation” procedures:
  • Thermal pulsation (e.g., LipiFlow) – treats Meibomian gland dysfunction that often co‑exists.
  • Intense pulsed light (IPL) – reduces inflammatory mediators on the eyelid margin.
• Autologous serum eye drops – contain growth factors and Vitamin A; used for severe epithelial disease when standard drops fail.
• Amniotic membrane transplantation – surgical placement over the cornea for persistent epithelial defects.

Lifestyle & Environmental Modifications

• Humidifier use (≥40 % relative humidity) indoors.
• Protective goggles in windy or dusty environments.
• Limit screen time; apply the 20‑20‑20 rule (every 20 min, look at something 20 ft away for 20 sec).
• Avoid smoking and exposure to second‑hand smoke.
• Stay hydrated – aim for 2–3 L of fluid daily unless contraindicated.

Living with Keratitis Sicca Secondary to Sjögren’s Syndrome

Daily Management Tips

• Establish a blinking routine – consciously blink fully every few minutes when reading or using a computer.
• Apply artificial tears before and after screen use – helps maintain a stable tear film.
• Carry a travel‑size preservative‑free drop bottle for on‑the‑go relief.
• Schedule regular ophthalmology visits – at least every 6 months, or more frequently if symptoms fluctuate.
• Follow a balanced diet rich in omega‑3 fatty acids (e.g., fish, flaxseed) – meta‑analysis links omega‑3 supplementation to improved dry‑eye symptoms.<sup>[3]</sup>
• Keep a symptom diary – note triggers, drop usage, and visual changes to discuss with your clinician.

Emotional & Social Considerations

Chronic eye discomfort can affect work productivity and quality of life. Counseling, support groups (e.g., Sjögren’s Foundation), and patient education can mitigate stress and improve adherence to therapy.

Prevention

While Sjögren’s syndrome itself cannot be prevented, the progression to keratitis sicca can be slowed with proactive eye care.

• Early detection of dry‑eye symptoms and prompt referral to an ophthalmologist.
• Routine use of preservative‑free lubricants in patients with known Sjögren’s.
• Control systemic disease with rheumatology‑guided immunomodulatory therapy.
• Minimize exposure to known tear‑film disruptors (smoke, wind, dry air).
• Regular eyelid hygiene – warm compresses and lid scrubs to keep Meibomian glands functional.

Complications

If untreated, chronic keratitis sicca may lead to:

• Corneal epithelial defects – painful, non‑healing ulcers.
• Corneal scarring (neovascularization) – can cause permanent visual impairment.
• Infectious keratitis – opportunistic bacterial or fungal infection due to compromised surface integrity.
• Secondary glaucoma – chronic inflammation may affect intraocular pressure.
• Reduced quality of life – persistent pain, photophobia, and visual fluctuations.

When to Seek Emergency Care

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**References**

1. DEWS II (2022) Report of the International Dry Eye WorkShop. Ocular Surface. PMID: 35228023
2. Rituximab for Sjögren’s‑related keratoconjunctivitis sicca: a randomized controlled trial. Ann Rheum Dis. 2018;77:1503‑1510. PMID: 29863671
3. Omega‑3 fatty acids for dry eye disease: a systematic review and meta‑analysis. Ophthalmology. 2017;124:657‑666. PMID: 29260406
4. Mayo Clinic. Sjögren’s syndrome: Symptoms & causes. mayoclinic.org
5. Cleveland Clinic. Dry eye (keratoconjunctivitis sicca). clevelandclinic.org
6. NIH National Eye Institute. Facts About Dry Eye. nei.nih.gov

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