How should dry eye symptoms be managed in a patient with Sjögren’s syndrome?

Management of Dry Eye in Sjögren's Syndrome

Start with preservative-free artificial tears containing methylcellulose or hyaluronate at least 4-6 times daily, escalating to hourly as needed, and add bedtime ophthalmic ointment for overnight symptom control. 1

Stepwise Treatment Algorithm

Step 1: First-Line Therapy (Artificial Tears + Ointment)

• Initiate preservative-free artificial tears containing methylcellulose or hyaluronate at minimum twice daily, escalating frequency up to hourly based on symptom severity 2, 1
• Critical caveat: If using preserved artificial tears, limit to maximum 4 applications daily to avoid additional ocular surface toxicity from preservatives 1
• Add ophthalmic lubricating ointment at bedtime for overnight symptom control, though this causes temporary blurred vision and requires morning lid hygiene to prevent blepharitis 2, 1

Step 2: Anti-Inflammatory Therapy (If No Response to Step 1)

• Add topical cyclosporine 0.05% twice daily when symptoms persist after maximizing artificial tear use, as it prevents T-cell activation and reduces inflammatory cytokine production 1
• Cyclosporine demonstrates success rates of 74% in mild, 72% in moderate, and 67% in severe dry eye, though approximately 17% of patients experience transient ocular burning 1
• Alternative consideration: A 2017 systematic review found that topical fluorometholone over 8 weeks was more effective than cyclosporine for dry eye symptoms and signs in Sjögren's syndrome 3

Step 3: Short-Term Rescue Therapy (For Refractory/Severe Cases)

Refractory disease is defined as failure to improve after maximizing artificial tears and ointments, after ruling out other ocular processes like blepharitis. 2

• Prescribe short-term topical corticosteroids (fluorometholone or loteprednol) for maximum 2-4 weeks to rapidly decrease ocular irritation and corneal staining 2, 1
• Critical pitfall: Never continue topical corticosteroids beyond 4 weeks due to risk of corneal-scleral melts, perforation, ulceration, infections, increased intraocular pressure, and cataract development 2, 1
• A 2022 study confirmed that prednisolone 1% had the strongest association with improvement of corneal staining scores when ocular surface inflammation is present 4

Step 4: Advanced Therapies (For Treatment-Refractory Disease)

Autologous serum eye drops:

• Use 20% concentration autologous serum eye drops to improve ocular irritation symptoms and corneal/conjunctival staining in severe Sjögren's-related dry eye 2, 1
• For patients where repeated blood sampling is not feasible, allogeneic serum eye drops are an effective and safe alternative 2

Tear conservation strategies:

• Trial temporary silicone punctal plugs first before considering permanent punctal cautery, as cautery is not readily reversible 2, 1
• A 2022 study demonstrated that punctal plug insertions in lower and upper eyelids were strongly associated with improvement of corneal staining scores (β = 2.70 and 1.80, p < 0.001), making this the mainstay treatment even in the presence of ocular surface inflammation 4
• Use stepwise approach: occlude no more than one punctum per eye at each treatment session 2

Oral secretagogues:

• Prescribe pilocarpine 5 mg orally four times daily or cevimeline to stimulate tear production, though efficacy is significantly greater for oral dryness than ocular symptoms 2, 1
• Pilocarpine improved ability to focus eyes during reading and symptoms of blurred vision (possibly related to miosis effect), but disappointingly showed no improvement in light sensitivity or ocular discomfort 2
• Common side effect: Excessive sweating occurs in over 40% of patients, with 2% withdrawing from treatment due to drug-related side effects 2
• Cevimeline may have fewer adverse systemic side effects than oral pilocarpine 2

Step 5: Specialized Interventions (For Severe Refractory Cases)

• Consider rigid gas-permeable scleral lenses for severe dry eye, though use may be limited by fitting difficulties, cost, and patient ability to manage insertion/removal 2
• For filamentary keratitis: Debride filaments with moistened cotton-tip applicator and apply topical N-acetylcysteine 10% to decrease mucinous tear film viscosity 2
• Limited tarsorrhaphy can decrease tear film instability in patients with severe dry eye who have not responded to other therapies 2

Critical Management Principles

Ophthalmology referral is essential for patients with refractory or severe ocular dryness, ideally to an ophthalmologist with substantial experience in corneal disease 2

Severity assessment should include:

• Ocular Staining Score (OSS) measurement, with OSS ≥5 indicating severity 2
• Ocular Surface Disease Index (OSDI) for patient symptoms 2
• Additional severity criteria: impaired visual function (photophobia, visual acuity modification), blepharospasm secondary to ocular inflammation, or severe meibomian gland disease 2

What NOT to do:

• Avoid anti-TNF biologics as they have been proven ineffective in Sjögren's syndrome 2, 5
• Do not expect hydroxychloroquine (commonly prescribed for fatigue and arthralgias) to improve dry eye symptoms, as literature does not demonstrate strong evidence for efficacy in ocular dryness 2, 5
• Never use topical NSAIDs long-term due to risk of corneal-scleral melts, perforation, ulceration, and severe keratopathy 2