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

Treatment for Systemic Lupus Erythematosus (SLE)

Hydroxychloroquine is the cornerstone medication for all patients with SLE and should be prescribed for every patient unless absolutely contraindicated. 1, 2, 3

First-Line Treatment Approach

Antimalarials

• Hydroxychloroquine 200-400 mg daily (standard dose)
  • Requires baseline and periodic ophthalmologic examinations to monitor for retinal toxicity
  • Can be taken with food or milk to improve tolerability
  • Should be continued during pregnancy 1

Glucocorticoids

• Use at the lowest possible dose for the shortest period of time 4
• Dosing based on severity:
  • Mild manifestations: Prednisone ≤0.5 mg/kg/day
  • Moderate manifestations: Prednisone 0.5-1 mg/kg/day
  • Severe manifestations: Methylprednisolone pulses (500-1000 mg IV for 3 days) 1

Organ-Specific Treatment Approaches

Cutaneous Lupus

1. First-line: Topical agents + hydroxychloroquine ± glucocorticoids
2. Second-line: Azathioprine
3. Third-line: Mycophenolate mofetil 5

Musculoskeletal Manifestations

1. First-line: Hydroxychloroquine ± glucocorticoids
2. Second-line: Methotrexate
3. Third-line: Rituximab 5

Lupus Nephritis

1. Induction therapy:
   • Class III/IV: Methylprednisolone pulses followed by oral prednisone with mycophenolate mofetil (1-3 g/day) or cyclophosphamide
   • Class V: Prednisone with mycophenolate mofetil
   • High-risk: High-dose intravenous cyclophosphamide 1, 4
2. Maintenance therapy:
   • Mycophenolate mofetil or azathioprine at lower doses 4
   • Consider belimumab (FDA-approved for lupus nephritis) 6

Neuropsychiatric Lupus

1. First-line: Glucocorticoids and cyclophosphamide
2. Maintenance: Azathioprine or mycophenolate mofetil
3. Refractory cases: Rituximab, IVIG, or plasmapheresis 5

Hematological Manifestations

1. First-line: Glucocorticoids (dose based on severity)
2. Second-line: Mycophenolate mofetil or cyclophosphamide
3. Refractory cases: Rituximab (particularly effective for thrombocytopenia or hemolytic anemia) 1

Cardiopulmonary Manifestations

• Pericarditis:

  1. First-line: NSAIDs, then glucocorticoids ± hydroxychloroquine
  2. Second-line: Azathioprine, mycophenolate, or methotrexate
  3. Refractory cases: Belimumab or rituximab 5

• Interstitial lung disease:

  1. Induction: Glucocorticoids with mycophenolate or cyclophosphamide
  2. Refractory cases: Rituximab or IVIG 5

Newer Biologic Therapies

Belimumab

• FDA-approved for active SLE in patients ≥5 years receiving standard therapy
• Also approved for lupus nephritis
• Not recommended for severe active CNS lupus 6, 3

Other Biologics

• Anifrolumab: FDA-approved for active SLE 3
• Voclosporin: FDA-approved for lupus nephritis 3
• Rituximab: Consider for refractory disease, particularly effective for hematologic manifestations 1, 5

Monitoring and Follow-up

• Disease activity assessment:

  • Every 1-3 months during active disease
  • Every 6-12 months once stable 1

• Laboratory monitoring:

  • Complete blood count
  • Renal function tests and urinalysis
  • Complement levels (C3, C4)
  • Anti-dsDNA antibodies 1

Prevention of Complications

1. Osteoporosis prevention:

   • Calcium and vitamin D supplementation
   • Minimize glucocorticoid exposure 1

2. Cardiovascular risk management:

   • Blood pressure control (target <130/80 mmHg)
   • Lipid management (target LDL <100 mg/dl)
   • Smoking cessation 1

3. Infection prevention:

   • Appropriate vaccinations
   • Prophylaxis when indicated based on immunosuppression level 1

4. Sun protection:

   • UV light avoidance
   • Sunscreen use 1

Common Pitfalls to Avoid

1. Delaying hydroxychloroquine initiation
2. Overreliance on glucocorticoids
3. Inadequate monitoring for drug toxicity
4. Missing concurrent infections
5. Failing to address cardiovascular risk factors 1

The therapeutic goal should be to reach and maintain remission or low disease activity as soon as possible after diagnosis and for as long as possible, while minimizing medication-related adverse effects 4, 3.