What is the recommended treatment for lupus?

Treatment for Lupus

Hydroxychloroquine is the cornerstone medication recommended for all patients with systemic lupus erythematosus (SLE), as it reduces disease flares, morbidity, and mortality. 1, 2, 3

First-Line Treatment

• Hydroxychloroquine (HCQ):
  • Dosage: 200-400 mg daily 1, 4
  • Benefits: Reduces disease flares, damage accrual, and mortality 5
  • Monitoring: Baseline and periodic ophthalmologic examinations to screen for retinal toxicity 1
  • Caution: Do not exceed 5 mg/kg actual body weight to minimize risk of retinopathy 4

Treatment Based on Disease Manifestations

Mild Disease (Constitutional, Mucocutaneous, Musculoskeletal)

1. Hydroxychloroquine 200-400 mg daily 4
2. Low-dose glucocorticoids (0.25-0.5 mg/kg/day), targeting the lowest effective dose (≤5 mg/day) for the shortest duration possible 1
3. NSAIDs for symptomatic relief of arthritis and myalgia 2

Moderate Disease

1. Continue hydroxychloroquine
2. Add immunosuppressants based on predominant manifestations:
   • Musculoskeletal: Methotrexate (5-15 mg weekly) or leflunomide 1
   • Milder disease: Azathioprine (1-2.5 mg/kg daily) 1
   • Alternative: Mycophenolate mofetil (0.5-1 g twice daily) if better tolerated than azathioprine 1

Lupus Nephritis (Renal Involvement)

Treatment should be guided by renal biopsy findings 6:

1. Class III/IV (±V) Lupus Nephritis:

   • Methylprednisolone pulses followed by oral prednisone with either:
     • Mycophenolic acid (MPA) or
     • Low-dose intravenous cyclophosphamide 6, 1
   • For high-risk features: Higher doses of cyclophosphamide 6

2. Pure Class V Lupus Nephritis with nephrotic-range proteinuria:

   • Mycophenolate mofetil with oral glucocorticoids 6, 1

3. Maintenance therapy:

   • Continue immunosuppression with MPA or azathioprine for at least 3 years 6
   • If initial treatment was MPA, continue with MPA for maintenance 6

Refractory Disease

For patients not responding to standard therapy:

1. Switch between agents (if MPA fails, try cyclophosphamide or vice versa) 6
2. Consider biologics:
   • Belimumab: Approved for active SLE and lupus nephritis in patients ≥5 years old 7, 3
   • Rituximab: Consider for refractory cases 1
   • Anifrolumab: Approved for active SLE 3
   • Voclosporin: Approved for lupus nephritis 3

Monitoring and Treatment Goals

• Treatment goal: Complete renal response (proteinuria <0.5 g/24h with normal or near-normal renal function) 6
• Disease activity assessment: SLEDAI-2K score to measure disease activity across 9 organ systems 1
• Regular monitoring:
  • Complete blood count every 1-3 months during active disease
  • Anti-dsDNA antibodies and complement levels
  • Serum creatinine, urinalysis, and urine protein/creatinine ratio for renal involvement 1

Special Considerations

Pregnancy

• Hydroxychloroquine can be continued during pregnancy 1
• Avoid retinoids and thalidomide due to teratogenic effects 1
• Switch to appropriate medications without reducing treatment intensity 6

Cardiovascular Risk Management

• Blood pressure control (target <130/80 mmHg)
• Manage dyslipidemia 1

Non-Pharmacological Interventions

• Physical therapy and exercise once acute crisis stabilizes 1
• Aerobic exercise programs to reduce fatigue and depressive symptoms 1
• Patient education and support for self-management 1
• Photoprotection to prevent flares 1

Common Pitfalls to Avoid

1. Undertreatment: Not using hydroxychloroquine in all SLE patients without contraindications
2. Overtreatment: Using high-dose glucocorticoids (>5 mg/kg/day) for prolonged periods, increasing risk of infections and damage accrual 5
3. Inadequate monitoring: Failing to regularly assess disease activity and medication toxicity
4. Discontinuing hydroxychloroquine: Maintaining HCQ therapy is critical for reducing flares and mortality
5. Delayed treatment of nephritis: Renal biopsy should be performed promptly for any sign of renal involvement 6

For severe active central nervous system lupus, belimumab is not recommended 7.