What are the treatment options for Sjogren's syndrome?

Treatment of Sjögren's Syndrome

The treatment of Sjögren's syndrome should follow a logical sequence starting with symptomatic management of dryness symptoms using topical therapies, followed by systemic treatments tailored to specific organ involvement and disease severity. 1

General Approach

• Management requires a multidisciplinary approach with specialists in autoimmune diseases coordinating care, based on shared decision-making between patient and healthcare team 1, 2
• Treatment should be tailored to specific manifestations (sicca symptoms vs. systemic disease) using standardized assessment tools 1, 2

Management of Sicca Symptoms

Oral Dryness

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

• Treatment approach should be based on salivary gland function severity:

  • For mild dysfunction: Non-pharmacological stimulation (sugar-free gum, frequent sips of water) 2, 3
  • For moderate dysfunction: Pharmacological stimulation with muscarinic agonists (pilocarpine or cevimeline) 1, 2
  • For severe dysfunction with no salivary output: Saliva substitution products 1, 3

• Pilocarpine is FDA-approved for Sjögren's syndrome at 5 mg four times daily, with efficacy established by 6 weeks of use 4

• Patients with moderate hepatic impairment should start at 5 mg twice daily 4

Ocular Dryness

• First-line therapy: Artificial tears containing methylcellulose or hyaluronate at least twice daily, with frequency increased as needed 1, 3

• Preservative-free formulations are recommended for patients requiring four or more applications per day 1

• Ophthalmic ointments can provide overnight symptom control 1

• For refractory/severe ocular dryness:

  • Topical immunosuppressive-containing drops (short-term use, maximum 2-4 weeks) 1
  • Autologous serum eye drops 1, 3
  • Patients should be managed by an ophthalmologist with experience in corneal disease 1

Management of Constitutional Symptoms

• Evaluate for concomitant conditions that may contribute to fatigue or pain 2, 3
• For musculoskeletal pain:
  • Use analgesics according to pain severity following the WHO pain ladder 3, 5
  • Consider short-term moderate-dose glucocorticoids for acute pain 3
  • Balance potential benefits against side effects 1

Management of Systemic Disease

• Treatment should be tailored to organ-specific severity using ESSDAI definitions 1, 2
• Follow a sequential approach based on severity:

Mild to Moderate Systemic Disease

• Hydroxychloroquine may be appropriate for patients with fatigue, arthralgia, and myalgia 6
• Glucocorticoids should be used at the minimum dose and length of time necessary to control active systemic disease 1, 3

Moderate to Severe Systemic Disease

• Immunosuppressive agents (cyclophosphamide, azathioprine, methotrexate, leflunomide, mycophenolate) should be used mainly as glucocorticoid-sparing agents 1, 2
• No evidence supports the choice of one immunosuppressive agent over another 1

Severe, Refractory Systemic Disease

• B-cell targeted therapies, particularly rituximab, may be considered 1, 2
• The systemic organ-specific therapeutic approach generally follows sequential (or combined) use of glucocorticoids, immunosuppressive agents, and biologics 1, 2

Special Considerations

• Treatment of B-cell lymphoma should be individualized according to the specific histological subtype and disease stage 1
• For low-grade lymphomas limited to exocrine glands without constitutional symptoms, a watchful waiting approach may be considered 1
• For disseminated MALT lymphoma or concomitant high disease activity, rituximab-based chemotherapy regimens are recommended 1

Common Pitfalls to Avoid

• Failing to distinguish between treatment of sicca symptoms versus systemic disease 2, 5
• Prolonged use of glucocorticoids without appropriate steroid-sparing strategies 2
• Overlooking the need for multidisciplinary care, especially for complex manifestations 2
• Continuing topical NSAIDs or corticosteroids beyond recommended duration (risk of corneal-scleral melts, infections, increased intraocular pressure) 1