Initial Management of Sjögren's Syndrome
The initial management of Sjögren's syndrome should focus on symptom relief through topical therapies for sicca symptoms, with muscarinic agonists as first-line pharmacological treatment for patients with residual gland function, followed by targeted approaches for systemic manifestations if present. 1, 2
Management of Sicca Symptoms
Dry Mouth Management
- For mild salivary gland dysfunction, begin with non-pharmacological stimulation including sugar-free gum and frequent sips of water 1, 3
- For moderate salivary gland dysfunction with residual function, use muscarinic agonists such as pilocarpine (5 mg four times daily) or cevimeline 3, 4
- For severe salivary gland dysfunction with no salivary output, provide saliva substitution products 1, 2
- Rigorous oral hygiene is essential to prevent infections and dental caries 2
Dry Eye Management
- First-line therapy for ocular dryness is artificial tears containing methylcellulose or hyaluronate at least twice daily 1, 3
- For persistent ocular dryness symptoms, consider ocular gels and ointments (especially for nighttime use) or autologous serum eye drops 3, 2
- Topical non-steroidal anti-inflammatory drugs and topical corticosteroids may be used for inflammatory flares 1
- Topical cyclosporine A can be considered for severe keratoconjunctivitis sicca 5
Management of Constitutional Symptoms
- Evaluate for contributing conditions for fatigue and musculoskeletal pain 1, 2
- For musculoskeletal pain, use analgesics according to pain severity following the WHO pain ladder 1, 3
- Consider short-term moderate-dose glucocorticoids for acute pain 1, 3
- Hydroxychloroquine may be appropriate for patients with fatigue, arthralgia, and myalgia 5
Management of Systemic Manifestations
Pulmonary Involvement
- Perform complete pulmonary function testing to assess severity of small airway disease 1, 3
- Obtain high-resolution CT imaging with expiratory views for small airway disease 1
- Treatment options for small airway disease include short course of systemic steroids, nebulized or inhaled bronchodilators and/or corticosteroids 1, 3
- For symptomatic/moderate-severe interstitial lung disease, first-line treatment is systemic corticosteroids at 0.5-1.0 mg/kg 1, 2
- First-line maintenance for interstitial lung disease is mycophenolate mofetil or azathioprine as steroid-sparing agents 1, 3
Sequential Treatment Approach Based on Disease Severity
- For mild disease with predominant sicca symptoms, use topical therapies and symptomatic management 1, 2
- For moderate disease, consider short-term glucocorticoids at minimum effective dose and duration 1, 2
- For severe disease with significant systemic manifestations, use glucocorticoids for initial control and immunosuppressive agents as steroid-sparing options 1, 3
- B-cell targeted therapies, particularly rituximab, may be considered for refractory disease 3, 6
Important Considerations and Pitfalls
- Clearly distinguish between treatment of sicca symptoms versus systemic disease 1, 2
- Avoid prolonged use of glucocorticoids without appropriate steroid-sparing strategies 1, 2
- Monitor for potential side effects of immunosuppressive medications 1, 2
- Smoking cessation is strongly recommended for all Sjögren's patients 1, 2
- Ensure vaccination against influenza and pneumococcal infection 1, 3
- When using pilocarpine, start with 5 mg four times daily; the most common side effects include sweating, urinary frequency, flushing, and chills 4, 7
- For patients with moderate hepatic impairment, the starting dose of pilocarpine should be 5 mg twice daily 4
- Regular assessment of disease activity using the ESSDAI index is crucial for guiding therapeutic decisions 2