What is the initial management for Sjögren's syndrome?

Initial Management of Sjögren's Syndrome

The first therapeutic approach for Sjögren's syndrome should be symptomatic relief of dryness using topical therapies, with a multidisciplinary approach coordinated at centers of expertise. 1

Overarching Principles

• Patients should be managed at or in close collaboration with centers of expertise following a multidisciplinary approach 1
• Symptomatic relief using topical therapies should be the first therapeutic approach for dryness 1
• Systemic therapies may be considered for active systemic disease 1

Management of Oral Dryness

1. Baseline evaluation of salivary gland function is recommended before starting treatment 1

2. Treatment approach based on salivary gland function: 1

   • Mild dysfunction: Non-pharmacological stimulation (sugar-free gum/lozenges, increased hydration)
   • Moderate dysfunction: Pharmacological stimulation with sialogogues
   • Severe dysfunction: Saliva substitution

3. Specific oral care measures: 2

   • Use bland oral rinse (1 teaspoon salt, 1 teaspoon baking soda in 4 cups of water) several times daily
   • Brush with ultra-soft toothbrush and prescription-strength fluoride toothpaste
   • Apply water-based lubricants after cleaning
   • Avoid commercial mouthwashes containing alcohol
   • Regular dental check-ups every 6 months

4. Pharmacological options for moderate-severe oral dryness:

   • Pilocarpine: 5 mg taken four times daily (FDA-approved dose for Sjögren's syndrome) 3
   • Cevimeline: 30 mg three times daily (fewer adverse effects than pilocarpine) 2

Management of Ocular Dryness

1. First-line approach: Artificial tears and ocular gels/ointments 1

2. For refractory/severe ocular dryness: 1

   • Topical immunosuppressive-containing drops
   • Autologous serum eye drops

3. Additional measures: 2

   • Avoid anticholinergic medications that can worsen sicca symptoms
   • Consider topical cyclosporine A for severe keratoconjunctivitis sicca

Management of Fatigue and Pain

1. Evaluate concomitant diseases in patients with fatigue/pain and score severity using specific tools 1

2. For musculoskeletal pain, consider analgesics or other pain-modifying agents, balancing benefits and side effects 1

3. For fatigue, arthralgia, and myalgia:

   • Hydroxychloroquine has been traditionally used but showed no significant benefit over placebo in a randomized controlled trial 4

Systemic Disease Management

• Treatment of systemic disease should be tailored to specific organ involvement 1
• For severe extraglandular manifestations, systemic immunosuppression may be required 5
• B-cell depleting therapies have shown promising results, while TNF-alpha inhibitors have demonstrated lack of efficacy 6, 5

Special Considerations

• Respiratory involvement: May require pulmonologist referral for complete pulmonary function testing and high-resolution CT imaging 2
• Lymphoproliferative complications: Refer to hematologist/oncologist if symptoms like unexplained weight loss, fevers, night sweats, or lymphadenopathy develop (5-18% increased risk of lymphoma) 2

Common Pitfalls to Avoid

• Delayed treatment: Efficacy for pilocarpine may require at least 6 weeks of use 3
• Inappropriate medication use: Avoid anticholinergic medications that worsen sicca symptoms 2
• Overlooking systemic manifestations: While focusing on sicca symptoms, don't miss potentially serious extraglandular involvement
• Overuse of immunosuppression: Traditional immunosuppressive agents have limited evidence for efficacy in primary Sjögren's syndrome 6, 7
• Using lemon-glycerin swabs: These produce acidic pH, dry oral tissues, cause enamel erosion, and exhaust salivary mechanisms 2