Sjögren's Syndrome
Sjögren's syndrome is the rheumatological condition most likely causing dry eyes and vision changes in a woman over 40. This autoimmune disorder affects the lacrimal and salivary glands through lymphocytic infiltration, resulting in the characteristic "sicca complex" of dry eyes (keratoconjunctivitis sicca) and dry mouth (xerostomia) 1, 2.
Why Sjögren's Syndrome Should Be Your Primary Consideration
Approximately 10% of patients with clinically significant aqueous deficient dry eye have underlying primary Sjögren's syndrome, making this a common presentation that warrants active screening 1, 2. The demographic profile fits perfectly—women are affected 20 times more frequently than men (20:1 ratio), and the condition predominantly affects postmenopausal women 2, 3.
Key Clinical Features to Identify
The presentation typically includes:
Ocular manifestations: Dry eyes with foreign body sensation, eye irritation, light sensitivity, and frequent need for eye drops 2. Vision changes occur due to tear film instability causing fluctuating vision, particularly worsening at the end of the day 1.
Oral manifestations: Dry mouth requiring liquids to swallow dry foods, frequent water sipping, burning mouth sensation, angular cheilitis, and increased dental cavities 2.
Systemic symptoms: Joint pain (arthralgias), muscle pain (myalgias), extreme fatigue, and vaginal dryness 2.
Diagnostic Approach
Ophthalmologists should maintain a high index of suspicion for Sjögren's syndrome and have a low threshold for serological work-up when encountering clinically significant dry eye 1.
Essential Serological Testing
Anti-SSA/Ro antibody: The most specific marker, scoring 3 points in diagnostic criteria 2, 3. However, negative anti-SSA does not exclude the diagnosis 4.
Rheumatoid factor (RF): Should be checked in all patients with clinically significant dry eye and dry mouth 1.
Additional markers: ANA, anti-SSB/La antibodies, and inflammatory markers (ESR, CRP) 2.
Objective Ocular Testing
Schirmer test without anesthesia: ≤5 mm/5 minutes scores 1 point 2.
Ocular surface staining: Abnormal ocular staining score ≥5 or van Bijsterveld score ≥4 scores 1 point 2. Use lissamine green or fluorescein to assess punctate epithelial erosions 1.
Tear film assessment: Evaluate tear meniscus height, tear break-up time, and tear film osmolarity 2.
Diagnostic Threshold
A total score of ≥4 points using the weighted scoring system meets criteria for primary Sjögren's syndrome 2. This can be achieved through various combinations of positive serological and objective findings.
Critical Management Considerations
Mandatory Rheumatology Referral
Co-management with a rheumatologist is essential, not optional 2, 4. This is critical because:
Approximately 5% of Sjögren's patients develop lymphoid malignancy, representing a 320 cases per 100,000 patient-years increased incidence 1, 2.
Decreased C4 levels at diagnosis are associated with higher lymphoma risk 2, 3.
Patients may develop serious systemic complications including vasculitis, peripheral neuropathy, interstitial lung disease, and renal involvement 2, 5.
Symptomatic Treatment
While awaiting rheumatology evaluation:
First-line therapy: Artificial tears and lubricating ointments for dry eyes; saliva substitutes for dry mouth 2, 4.
Moderate to severe dry eye: Topical cyclosporine 0.05% for inflammatory component 2, 4.
Systemic therapy: Hydroxychloroquine 200-400 mg daily may be considered for mild systemic manifestations, though evidence for dry eye improvement is weak 2.
Important Clinical Pitfalls
Don't dismiss the diagnosis in younger patients or men despite the strong female and postmenopausal predominance—the disease can occur in all demographics 1, 3.
Don't overlook medication-induced sicca symptoms as antihistamines, diuretics, antidepressants, and anticholinergics can mimic Sjögren's syndrome 2.
Don't ignore chronic cough with dry eyes—unexplained chronic cough led to Sjögren's diagnosis in 36% of previously undiagnosed patients 2.
Vision-Threatening Complications
Beyond dry eye, Sjögren's syndrome can cause serious extraglandular ocular manifestations including corneal melt/perforation, uveitis, scleritis, retinal vasculitis, and optic neuritis 6. Patients with severe dry eye and underlying inflammatory conditions may develop corneal scarring, thinning, neovascularization, and microbial or sterile corneal ulceration with possible perforation 1.