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

Treatment of Systemic Lupus Erythematosus (SLE)

Hydroxychloroquine is recommended for all patients with SLE, unless contraindicated, as the cornerstone of therapy regardless of disease manifestations, with additional treatments tailored based on organ involvement and disease severity. 1, 2

First-Line Therapy

• Hydroxychloroquine (HCQ):

  • Dosage: 200-400 mg/day (≤5 mg/kg/real body weight) 1, 2, 3
  • Benefits: Reduces flares, damage accrual, thrombotic events, and mortality 2
  • Monitoring: Baseline ophthalmologic exam, then after 5 years and yearly thereafter 1
  • Should be continued indefinitely in all patients 2

• Glucocorticoids (GCs):

  • Use at lowest possible dose and shortest duration 1
  • Initial therapy: Low-dose prednisone (≤7.5 mg/day) for mild disease 2
  • For flares: Adjust dose according to severity of organ involvement 1
  • Goal: Taper to ≤5 mg/day during maintenance phase 2

Treatment by Disease Severity

Mild Disease (Mucocutaneous, Musculoskeletal)

1. Hydroxychloroquine (mandatory)
2. Low-dose glucocorticoids
3. Consider adding methotrexate, leflunomide, or azathioprine if inadequate response 2

Moderate-to-Severe Disease

1. Hydroxychloroquine (mandatory)
2. Higher-dose glucocorticoids
3. Add immunosuppressant based on organ involvement:
   • Azathioprine
   • Mycophenolate mofetil (MMF)
   • Cyclophosphamide (for severe manifestations) 2, 4

Lupus Nephritis (Class III/IV/V)

1. Induction therapy (6 months):

   • Methylprednisolone pulses followed by oral prednisone
   • Plus either mycophenolate mofetil or low-dose cyclophosphamide 2
   • Tacrolimus is an alternative option 2

2. Maintenance therapy (at least 3 years):

   • Lower dose glucocorticoids (≤5 mg/day)
   • Mycophenolate mofetil or azathioprine 2
   • Continue hydroxychloroquine indefinitely

Neuropsychiatric Lupus

• First-line: Cyclophosphamide IV for CNS vasculitis
• Alternatives: Rituximab or mycophenolate mofetil for refractory cases 2

Biologic Therapies

• Belimumab:

  • FDA-approved for active SLE in patients ≥5 years receiving standard therapy 5
  • Also approved for active lupus nephritis 5, 4
  • Not recommended for severe active CNS lupus 5

• Anifrolumab: FDA-approved for active SLE 4

• Rituximab: Consider for refractory disease 2, 6

Adjunctive Treatments

• Antiphospholipid Antibody Positive Patients:

  • Anticoagulation or antiplatelet therapy 2

• Prevention Measures:

  • Osteoporosis prophylaxis: Calcium and vitamin D 2
  • Cardiovascular risk management: Statins for dyslipidemia (target LDL <100 mg/dl) 2
  • Infection prevention in immunosuppressed patients 2
  • Sun protection and UV light avoidance 2
  • Lifestyle modifications: Regular exercise, smoking cessation, weight control 2

Monitoring

• Disease activity: Every 1-3 months

  • Renal function, proteinuria
  • Complete blood count
  • Complement levels (C3, C4)
  • Anti-DNA antibodies 2

• Treatment response: Aim for remission or low disease activity in all organ systems 1, 4

Important Considerations

• Treatment goals include long-term patient survival, prevention of organ damage, and optimization of health-related quality of life 1

• Flares should be treated promptly by adjusting ongoing therapies or adding new agents based on severity 1

• Voclosporin is FDA-approved for lupus nephritis 4

• Pediatric SLE may require more aggressive initial therapy with careful monitoring of growth and development 6