Initial Treatment Approach for Sjögren's Syndrome
The first therapeutic approach to Sjögren's syndrome should focus on symptomatic relief of sicca symptoms using topical therapies, with muscarinic agonists as first-line pharmacological treatment for patients with residual gland function. 1, 2
Management of Sicca Symptoms
Ocular Dryness
- First-line therapy consists of artificial tears containing methylcellulose or hyaluronate at least twice daily 2, 3
- For persistent ocular dryness, consider autologous serum eye drops 2, 3
- Topical non-steroidal anti-inflammatory drugs and topical corticosteroids may be used for inflammatory flares 2
- Topical cyclosporine A may be considered for severe cases of keratoconjunctivitis sicca 4
Oral Dryness
- For mild salivary gland dysfunction, begin with non-pharmacological stimulation including sugar-free gum and frequent sips of water 2, 3
- For moderate salivary gland dysfunction with residual function, use muscarinic agonists (pilocarpine or cevimeline) to stimulate salivary flow 2, 3, 4
- For severe salivary gland dysfunction with no salivary output, provide saliva substitution products 2, 3
Management of Constitutional Symptoms
- Evaluate for contributing conditions for fatigue and musculoskeletal pain 2
- For musculoskeletal pain, use analgesics according to pain severity following the WHO pain ladder 2
- Consider hydroxychloroquine for patients with fatigue, arthralgia, and myalgia 4, 2
- Short-term moderate-dose glucocorticoids may be considered for acute pain 2, 3
Management of Systemic Manifestations
Treatment Approach Based on Disease Severity
- For mild disease with predominantly sicca symptoms, focus on topical therapies and symptomatic management 1, 2
- For moderate disease with constitutional symptoms, consider hydroxychloroquine and short-term glucocorticoids 2, 4
- For severe systemic disease, treatment should be tailored to organ-specific severity using the ESSDAI definitions 1, 2
Pharmacological Management of Systemic Disease
- Glucocorticoids should be used at the minimum dose and length of time necessary to control active systemic disease 1
- Immunosuppressive agents (azathioprine, mycophenolate mofetil) should be used mainly as glucocorticoid-sparing agents 1, 2
- B-cell targeted therapies, particularly rituximab, may be considered in patients with severe, refractory systemic disease 1, 2
Specific Organ Involvement
- For interstitial lung disease (ILD), first-line treatment is systemic corticosteroids (0.5-1.0 mg/kg) 1, 2
- First-line maintenance for ILD is mycophenolate mofetil or azathioprine as steroid-sparing agents 1, 2
- For small airway disease, consider short course of systemic steroids, nebulized/inhaled bronchodilators and/or corticosteroids 2
Important Considerations and Monitoring
- Smoking cessation is strongly recommended for all Sjögren's patients 2, 5
- Ensure vaccination against influenza and pneumococcal infection 5
- Regular assessment of disease activity using validated tools such as the ESSDAI is recommended 5
- Monitor for potential side effects of immunosuppressive medications 2, 3
- For patients with pulmonary involvement, repeat pulmonary function tests every 6-12 months 5
Common Pitfalls to Avoid
- Failing to distinguish between treatment of sicca symptoms versus systemic disease 2
- Prolonged use of glucocorticoids without appropriate steroid-sparing strategies 2
- Overlooking potential side effects of immunosuppressive medications 2
- Inadequate monitoring of disease progression, particularly in patients with organ involvement 5