What medications and treatment options are used for a patient with Systemic Lupus Erythematosus (SLE)?

Treatment Options for Systemic Lupus Erythematosus (SLE)

Hydroxychloroquine (HCQ) is the cornerstone therapy for all patients with SLE, and should be prescribed as the foundation of treatment, combined with targeted immunosuppressive agents based on organ involvement and disease severity. 1

First-Line Treatment Approach

• Hydroxychloroquine (HCQ):

  • Dose: 5 mg/kg real body weight per day
  • Benefits: Reduces mortality, disease flares, and organ damage
  • Should be continued long-term for all SLE patients 1

• Glucocorticoids (GCs):

  • Goal: Minimize dose to ≤7.5 mg/day prednisone equivalent or discontinue when possible
  • For acute flares: IV methylprednisolone pulses (250-1000 mg/day for 3 days) may be used 1
  • Prolonged use of doses >7.5 mg/day should be avoided due to risk of irreversible organ damage 1

Organ-Specific Treatment Approaches

Skin Disease

1. First-line: Topical agents (GCs, calcineurin inhibitors), antimalarials (HCQ), and/or systemic GCs 2
2. For non-responsive cases: Add methotrexate, retinoids, dapsone, or mycophenolate 2

Musculoskeletal Disease

1. For isolated, intermittent joint symptoms: Short courses of NSAIDs (with caution due to potential renal effects) 3
2. For more severe or recurrent symptoms: Low-dose corticosteroids (≤10 mg/day) with antimalarials 3
3. For refractory arthritis: Methotrexate in combination with antimalarials 3

Renal Disease (Lupus Nephritis)

1. Early recognition and diagnostic renal biopsy are essential 2
2. Induction therapy:
   • Mycophenolate mofetil (target 2-3 g/day) or low-dose IV cyclophosphamide 2, 1
   • For high-risk patients (reduced GFR, fibrous crescents, fibrinoid necrosis): High-dose IV cyclophosphamide 2
   • Newer options: Belimumab plus either MMF or cyclophosphamide, or MMF plus voclosporin (23.7 mg twice daily) 1
3. Maintenance therapy:
   • Mycophenolate mofetil or azathioprine 2, 1
   • Continue for ≥36 months with monitoring 1

Hematological Disease

1. Acute treatment of thrombocytopenia: High-dose GCs (including IV methylprednisolone pulses) and/or intravenous immunoglobulin G 2
2. Maintenance: Immunosuppressive/GC-sparing agents such as mycophenolate, azathioprine, or cyclosporine 2
3. Refractory cases: Rituximab or cyclophosphamide 2

Neuropsychiatric Disease

1. Proper attribution to SLE vs. non-SLE causes is essential 2
2. For inflammatory manifestations: Glucocorticoids/immunosuppressive agents 2
3. For atherothrombotic/antiphospholipid antibody-related manifestations: Antiplatelet/anticoagulants 2

Biologic Therapies

Belimumab

• Indicated for patients with inadequate response to standard therapy 2
• FDA-approved for active SLE and lupus nephritis 4
• Should not be administered with live vaccines 5
• For severe SLE: Continue through surgery if patient requires surgery 2

Rituximab

• Consider for organ-threatening disease refractory to standard immunosuppressives 2
• For severe SLE requiring surgery: Plan surgery in the last month of the dosing cycle 2

Anifrolumab

• Recently approved for active SLE 4
• For severe SLE: Continue through surgery if patient requires surgery 2

Treatment for Refractory Disease

For patients not responding to standard therapy (defined as residual disease activity not allowing tapering of glucocorticoids and/or frequent relapses):

1. Add belimumab to standard therapy 2
2. For organ-threatening disease refractory to standard immunosuppressives: Consider rituximab 2
3. For refractory cases: Consider leflunomide, abatacept, or in exceptional cases, anti-TNF antibodies 3

Special Considerations

• Pregnancy: HCQ, azathioprine, low-dose prednisone, and low-dose aspirin are compatible during pregnancy; mycophenolate mofetil should be discontinued ≥6 weeks before conception 1
• Surgery: For non-severe SLE, withhold mycophenolate, azathioprine, cyclosporine, mizoribine, tacrolimus, belimumab, and rituximab before surgery; for severe SLE, continue these medications through surgery 2
• Vaccination: Live vaccines should be avoided in patients receiving immunosuppressive therapy, particularly belimumab 5

Common Pitfalls to Avoid

1. Inadequate hydroxychloroquine use (cornerstone of therapy)
2. Excessive glucocorticoid exposure (>7.5 mg/day for prolonged periods)
3. Premature treatment changes before allowing adequate time for response
4. Overlooking comorbidities that may affect treatment choices
5. Inappropriate cyclophosphamide use in women and men of reproductive age due to gonadotoxicity 1

Remember that the treatment goal is to achieve remission or low disease activity in all organ systems, with regular monitoring for disease activity and medication side effects.