How is suspected Sjögren’s syndrome in a middle‑aged woman with chronic dry eyes and dry mouth diagnosed and managed?

Diagnosis and Management of Suspected Sjögren's Syndrome

Immediate Diagnostic Confirmation

For a middle-aged woman presenting with chronic dry eyes and dry mouth, proceed directly with serological testing for anti-SSA/Ro and anti-La/SSB antibodies, followed by objective ocular and salivary assessments to confirm Sjögren's syndrome diagnosis using the ACR/EULAR classification criteria requiring ≥4 points. 1

Initial Serological Workup

• Order anti-SSA/Ro antibody testing immediately, as positivity scores 3 points toward diagnosis and is present in the majority of Sjögren's patients 1
• Include anti-La/SSB antibodies, rheumatoid factor (RF), and antinuclear antibody (ANA) in the initial panel 2, 1
• Measure complement C4 levels at diagnosis, as decreased C4 is associated with significantly higher lymphoma risk (approximately 5% lifetime risk) 2, 3, 1

Objective Ocular Assessment

• Perform Schirmer test without anesthesia; ≤5 mm/5 minutes scores 1 point toward diagnosis 1
• Conduct ocular surface staining with lissamine green or fluorescein; an ocular staining score ≥5 or van Bijsterveld score ≥4 scores 1 point 1
• Evaluate tear break-up time, tear meniscus height, and document punctate epithelial erosions, mucous strands, and conjunctival hyperemia on slit-lamp examination 2, 1

Salivary Gland Evaluation

• Measure unstimulated whole salivary flow rate; ≤0.1 ml/minute scores 1 point 1
• Consider minor salivary gland biopsy if clinical suspicion remains high despite negative serology, looking for focal lymphocytic sialadenitis with focus score ≥1 foci/4 mm² (scores 3 points) 1

Mandatory Rheumatology Referral

All patients meeting diagnostic criteria for Sjögren's syndrome require immediate co-management with a rheumatologist due to the 5% lifetime risk of lymphoma and potential for life-threatening systemic complications including vasculitis and lymphoproliferative disorders. 2, 3, 1

• The rheumatologist will coordinate overall disease management, initiate systemic immunosuppressive therapy if needed, and monitor for lymphoma development 3, 1
• This referral is mandatory, not optional, given the significant mortality risk from systemic involvement 2, 4

Immediate Symptomatic Management

Ocular Dryness

• Prescribe preservative-free artificial tears for frequent use throughout the day as first-line therapy 1
• Add topical cyclosporine 0.05% twice daily for moderate to severe dry eye with inflammatory component 1
• Consider lubricating ointments at bedtime for overnight protection 1

Oral Dryness

• Recommend saliva substitutes as first-line symptomatic relief 1
• Prescribe pilocarpine 5 mg orally three to four times daily if inadequate response to topical measures 1
• Emphasize rigorous dental hygiene to prevent dental caries and gum disease, which occur at accelerated rates in xerostomia 2, 5

Screening for Systemic Complications

Pulmonary Evaluation

• Screen for chronic cough (>8 weeks duration), which affects approximately 38% of Sjögren's patients and may be the presenting symptom in previously undiagnosed cases 3, 1
• If respiratory symptoms present, order high-resolution CT chest with expiratory views, baseline pulmonary function tests, and oximetry at rest and with exercise 1
• Monitor for progressive dyspnea, as interstitial lung disease prevalence increases to 20% after 5 years 3

Lymphoma Surveillance

• Establish baseline complete blood count with differential to monitor for cytopenias 3
• Educate patients on red flag symptoms: unexplained weight loss, fevers, night sweats, or new lymphadenopathy (particularly head and neck region) 1
• Patients with decreased C4 levels at diagnosis require heightened surveillance given their elevated lymphoma risk 2, 3, 1

Other Autoimmune Screening

• Screen for thyroid dysfunction or autoimmune thyroiditis, which commonly coexists with primary Sjögren's syndrome 2, 4
• Evaluate for secondary Sjögren's syndrome by assessing for rheumatoid arthritis (joint deformities, ulnar deviation), scleroderma, or systemic lupus erythematosus 2, 1

Critical Pitfalls to Avoid

• Do not attribute dry eyes and mouth solely to medications (antihistamines, diuretics, antidepressants) without ruling out Sjögren's syndrome, especially in middle-aged women 1
• Do not delay rheumatology referral even if symptoms seem mild, as systemic complications can develop insidiously 3, 1
• Do not overlook chronic cough as a potential presenting symptom; 36% of previously undiagnosed Sjögren's patients were identified through unexplained chronic cough with dry eyes 3
• Do not fail to measure C4 levels at diagnosis, as this identifies patients at highest risk for lymphoma who require more intensive monitoring 2, 3, 1

Classification Criteria Summary

The diagnosis requires ≥4 points from the following weighted scoring system 1:

• Anti-SSA/Ro antibody positive: 3 points
• Focal lymphocytic sialadenitis (focus score ≥1 foci/4 mm²): 3 points
• Ocular staining score ≥5 or van Bijsterveld score ≥4: 1 point
• Schirmer test ≤5 mm/5 minutes: 1 point
• Unstimulated salivary flow ≤0.1 ml/minute: 1 point