What is the treatment for Systemic Lupus Erythematosus (SLE)?

Treatment of Systemic Lupus Erythematosus (SLE)

Hydroxychloroquine (200-400 mg/day) should be prescribed for all SLE patients regardless of disease manifestations, unless contraindicated, as the cornerstone of therapy. 1 Additional treatments should be tailored based on organ involvement and disease severity.

First-Line Treatment Approach

• Hydroxychloroquine: 200-400 mg daily (≤5 mg/kg real body weight) 1, 2

  • FDA-approved for SLE treatment in adults 2
  • Reduces disease activity, morbidity, and mortality 3
  • Should be continued indefinitely, even during remission 1

• Glucocorticoids: Use at lowest possible dose and shortest duration

  • Initial therapy: Low-dose prednisone (≤7.5 mg/day) 1
  • Goal: Taper to ≤5 mg/day during maintenance phase 1
  • Caution: Monitor for adverse effects including osteoporosis, hypertension, and diabetes

Treatment Based on Disease Severity

Mild Disease (Mucocutaneous, Musculoskeletal)

1. Hydroxychloroquine 200-400 mg daily
2. Low-dose glucocorticoids if needed
3. Add methotrexate, leflunomide, or azathioprine for inadequate response 1

Moderate-to-Severe Disease

1. Hydroxychloroquine (continue throughout treatment)
2. Higher-dose glucocorticoids initially
3. Add immunosuppressants based on organ involvement:
   • Azathioprine
   • Mycophenolate mofetil
   • Cyclophosphamide (for severe manifestations)

Organ-Specific Treatment

Lupus Nephritis

• Class III/IV: Methylprednisolone pulses followed by oral prednisone with mycophenolate mofetil or cyclophosphamide 1
• Class V: Prednisone with mycophenolate mofetil 1
• High-risk: High-dose intravenous cyclophosphamide 1
• Belimumab: FDA-approved for active lupus nephritis in patients ≥5 years receiving standard therapy 4

CNS Lupus

• First-line: Intravenous cyclophosphamide 1
• Alternatives: Rituximab or mycophenolate mofetil for refractory disease 1
• Note: Belimumab is not recommended for severe active CNS lupus 4

Newer Biologic Therapies

• Belimumab: FDA-approved for active SLE in patients ≥5 years receiving standard therapy 4, 3
• Anifrolumab: Recently approved for active SLE 3
• Voclosporin: Recently approved for lupus nephritis 3

Maintenance Therapy

After achieving remission:

1. Continue hydroxychloroquine indefinitely 1
2. Taper glucocorticoids to ≤5 mg/day prednisone 1
3. Maintenance with mycophenolate mofetil or azathioprine for at least 3 years 1

Monitoring and Supportive Care

• Monitor disease activity every 1-3 months 1

  • Renal function, proteinuria, complete blood count
  • Complement levels, anti-DNA antibodies

• Preventive measures:

  • Calcium and vitamin D supplementation for osteoporosis prevention 1
  • Management of cardiovascular risk factors (hypertension, dyslipidemia, diabetes) 1
  • Sun protection (UV light avoidance) 1
  • Regular exercise, smoking cessation, weight control 1

Treatment Goals

• Achieve remission or low disease activity in all organ systems 1, 3
• Prevent organ damage and optimize quality of life 1
• Minimize glucocorticoid exposure 1
• Treat flares promptly by adjusting therapies based on severity 1

Common Pitfalls to Avoid

1. Discontinuing hydroxychloroquine: Should be continued indefinitely even during remission
2. Prolonged high-dose glucocorticoid use: Aim to taper to ≤5 mg/day as soon as possible
3. Inadequate monitoring: Regular assessment of disease activity and medication side effects is essential
4. Neglecting comorbidities: Cardiovascular disease, osteoporosis, and infections require active prevention and management
5. Delayed treatment of flares: Prompt intervention is crucial to prevent organ damage

The treatment of SLE requires a comprehensive approach with careful consideration of disease manifestations, severity, and individual patient factors to optimize outcomes and minimize treatment-related complications.