What is the first line treatment for Sjogren's syndrome?

First-Line Treatment for Sjögren's Syndrome

The first-line treatment for Sjögren's syndrome is symptomatic relief using topical therapies for dryness symptoms, with the specific approach tailored to the type and severity of glandular dysfunction. 1

Management of Sicca Symptoms

Ocular Dryness

1. First-line therapy: Artificial tears and ocular gels/ointments containing methylcellulose or hyaluronate at least twice daily, increasing frequency as needed 1

   • Use preservative-free formulations for patients requiring ≥4 applications daily
   • Ophthalmic ointments can be used before bedtime for overnight symptom control

2. For refractory/severe ocular dryness:

   • Topical immunosuppressive-containing drops (e.g., cyclosporin A) for short-term use (2-4 weeks) 1
   • Autologous serum eye drops for cases not responding to other therapies

Oral Dryness

Treatment should be selected based on salivary gland function assessment:

1. For mild dysfunction: Non-pharmacological stimulation (sugar-free gum, frequent sips of water)
2. For moderate dysfunction: Pharmacological stimulation with muscarinic agonists:
   • Pilocarpine (FDA-approved for Sjögren's syndrome) 2
   • Cevimeline (FDA-approved for Sjögren's syndrome) 3
3. For severe dysfunction: Saliva substitution products 1

Management of Systemic Manifestations

For patients with systemic disease (defined by ESSDAI score ≥1), treatment should be tailored to organ-specific severity:

1. For fatigue and musculoskeletal pain:

   • Evaluate concomitant diseases and score severity using specific tools 1
   • Consider hydroxychloroquine for fatigue, arthralgia, and myalgia 4
   • Analgesics or pain-modifying agents for musculoskeletal pain, carefully weighing benefits against side effects 1

2. For moderate to severe systemic disease:

   • Glucocorticoids at minimum effective dose and duration necessary to control active disease 1
   • Immunosuppressive agents (primarily as steroid-sparing agents):
     • First-line maintenance: Mycophenolate mofetil (MMF) or azathioprine 1, 5
     • Second-line options: Rituximab for severe, refractory systemic disease 1

3. For interstitial lung disease (ILD):

   • Initial treatment: Systemic steroids (0.5-1.0 mg/kg) especially for organizing pneumonia 1
   • Maintenance therapy: MMF or azathioprine when long-term steroid-sparing therapy is required 1

Important Considerations and Pitfalls

• Avoid prolonged use of topical NSAIDs or corticosteroids for ocular symptoms due to risk of serious adverse effects (corneal melting, infections, increased intraocular pressure) 1

• Monitor for medication side effects:

  • Azathioprine: Test for thiopurine methyltransferase activity/genotype before initiating to prevent severe leukopenia 1
  • MMF: Monitor for nausea, diarrhea, hepatotoxicity, and bone marrow suppression 1
  • Glucocorticoids: Watch for glucose intolerance, avascular necrosis, fluid retention, hypertension, and other common steroid side effects 1

• Ineffective treatments to avoid:

  • TNF-alpha inhibitors have shown lack of efficacy in controlled trials 4, 6

• Multidisciplinary approach is essential with specialists in autoimmune diseases coordinating care, and involvement of ophthalmologists, dentists, and other specialists as needed 1

Remember that while symptomatic treatment can improve quality of life, no therapeutic intervention has been shown to reverse glandular dysfunction or cure sicca symptoms 1. Treatment goals should focus on symptom management and preventing progression of systemic disease.