Treatment Options for Sjögren's Syndrome
Treatment for Sjögren's syndrome should be tailored to specific symptoms and organ involvement, with topical therapies for sicca symptoms and systemic therapies reserved for patients with active systemic disease. 1, 2
Management of Sicca Symptoms
Ocular Dryness
- First-line therapy includes artificial tears containing methylcellulose or hyaluronate at least twice daily, with frequency increased as needed based on symptoms 1
- Preservative-free formulations are recommended for patients requiring four or more applications per day 1
- Ophthalmic ointments can be used before bedtime to provide overnight symptom control 1
- For refractory/severe ocular dryness, short-term (2-4 weeks) topical immunosuppressive therapy may be considered 1
- Topical cyclosporine A is recommended for patients who don't respond to artificial tears 1, 2
- Autologous serum eye drops may be used for severe cases unresponsive to other treatments 3
Oral Dryness
- Non-pharmacological approaches include sugar-free gum and frequent sips of water 3
- For moderate salivary gland dysfunction, muscarinic agonists like pilocarpine (5 mg four times daily) or cevimeline can increase salivary flow 3, 4
- Saliva substitution products are recommended for patients with no salivary output 3
- FDA-approved pilocarpine dosing for Sjögren's syndrome is 5 mg taken four times daily, with efficacy established by 6 weeks of use 4
Management of Airway Disease
- For patients with bronchiolitis, a trial of inhaled corticosteroids with or without macrolides is recommended 1
- Patients with clinically relevant bronchiectasis should be treated with:
- For dry, nonproductive cough, empirical humidification, secretagogues, and guaifenesin may be considered after excluding other causes 2
Management of Constitutional Symptoms
- For fatigue and musculoskeletal pain, evaluate for contributing conditions and use analgesics according to pain severity following the WHO pain ladder 3
- Hydroxychloroquine may be considered for fatigue and arthralgias, though evidence for efficacy is limited 2, 5
- For chronic, non-inflammatory pain, physical activity and aerobic exercise are recommended rather than medications 1
- Antidepressants and anticonvulsants may be considered for chronic musculoskeletal pain 1
- For chronic neuropathic pain, gabapentin, pregabalin, or amitriptyline may be used (with attention to potential exacerbations of dryness) 1
Management of Systemic Disease
- Systemic therapies should be restricted to patients with active systemic disease 1
- Treatment should be tailored to organ-specific severity using the EULAR Sjögren's Syndrome Disease Activity Index (ESSDAI) definitions 1, 3
- For moderate to severe systemic disease:
- Glucocorticoids should be used at the minimum effective dose and duration 3
- Immunosuppressive agents (azathioprine, mycophenolate, methotrexate) can be used as glucocorticoid-sparing agents 1, 3
- For interstitial lung disease, first-line maintenance includes mycophenolate mofetil or azathioprine 3
- B-cell targeted therapies, particularly rituximab, may be considered for severe, refractory systemic disease 3, 6
Monitoring and Follow-up
- Regular assessment of disease activity using validated tools such as the ESSDAI is recommended 2, 6
- For patients with pulmonary involvement, repeat pulmonary function tests every 6-12 months 2
- Monitor for development of lymphoma, which occurs in 2-5% of Sjögren's patients 6
Common Pitfalls to Avoid
- Using opioids for chronic pain management (not recommended) 1
- Failing to distinguish between treatment approaches for sicca symptoms versus systemic disease 3
- Using anticholinergics in patients with airway disorders, as they may worsen dryness of secretions 1
- Continuing use of topical NSAIDs long-term, which may lead to corneal-scleral melts, perforation, ulceration, and severe keratopathy 1