What is the treatment for lupus?

Treatment of Lupus

The cornerstone of lupus treatment is hydroxychloroquine for all patients, combined with glucocorticoids and specific immunosuppressive agents based on organ involvement and disease severity. 1

General Treatment Approach

First-Line Therapy

• Hydroxychloroquine: 200-400 mg daily (5 mg/kg real body weight) for all lupus patients 2, 1
  • Reduces disease flares, organ damage, and mortality
  • Should be continued long-term even during remission

Glucocorticoids

• Initial therapy for active disease:
  • IV methylprednisolone 250-500 mg/day for 1-3 days for severe flares 1
  • Oral prednisone 0.35-1.0 mg/kg/day (not exceeding 80 mg/day) 3
  • Goal: Taper to ≤7.5 mg/day within 3-6 months 1

Organ-Specific Treatment

Lupus Nephritis (Class III/IV)

1. Induction therapy (choose one option) 3:

   • Mycophenolic acid analogs (MMF 1.0-1.5 g twice daily)
   • Low-dose IV cyclophosphamide (500 mg every 2 weeks × 6)
   • Belimumab plus either MMF or cyclophosphamide
   • MMF plus calcineurin inhibitor (voclosporin 23.7 mg twice daily)

2. Maintenance therapy:

   • Continue induction agent at lower dose for ≥36 months 3
   • Monitor for complete response: proteinuria <0.5 g/g and stable kidney function 3

Neuropsychiatric Lupus

• Treatment depends on underlying mechanism 3:
  • Inflammatory: Glucocorticoids plus immunosuppressants
  • Thrombotic/ischemic: Anticoagulation/antithrombotic therapy if antiphospholipid antibodies present
  • Consider combination therapy when mechanisms coexist

Hematological Manifestations

• Thrombocytopenia (<30,000/mm³) 3:

  • Moderate/high-dose glucocorticoids plus immunosuppressant (AZA, MMF, or cyclosporine)
  • Consider IVIG for acute phase or inadequate response
  • Rituximab for refractory cases

• Autoimmune hemolytic anemia:

  • Similar approach as thrombocytopenia

Treatment for Non-Organ-Threatening Disease

• Musculoskeletal/cutaneous manifestations:
  • Hydroxychloroquine plus low-dose glucocorticoids 1
  • Add methotrexate or azathioprine if inadequate response
  • Topical agents (glucocorticoids, calcineurin inhibitors) for skin lesions

Monitoring and Treatment Adjustment

• Assess response using validated disease activity indices 3
• For inadequate response 3:
  1. Verify medication adherence
  2. Check drug levels if applicable
  3. Consider repeat biopsy if concern for chronicity
  4. Switch to alternative regimen for persistent active disease
  5. Consider rituximab, extended cyclophosphamide, or clinical trials for refractory disease

Special Considerations

Pregnancy

• Compatible medications 3:
  • Hydroxychloroquine, azathioprine, low-dose prednisone, low-dose aspirin
  • Discontinue mycophenolate mofetil ≥6 weeks before conception 3
  • Avoid cyclophosphamide and methotrexate

Antiphospholipid Syndrome

• Low-dose aspirin for primary prevention 3
• Long-term anticoagulation for secondary prevention of thrombosis 3
• Combined heparin and aspirin during pregnancy 3

Common Pitfalls to Avoid

1. Inadequate hydroxychloroquine use: Should be prescribed for all patients unless contraindicated
2. Excessive glucocorticoid exposure: Aim to taper to ≤7.5 mg/day within 3-6 months
3. Premature treatment changes: Allow 6-12 months for full response before changing therapy
4. Overlooking comorbidities: Monitor and manage infections, osteoporosis, and cardiovascular disease
5. Inappropriate cyclophosphamide use: Use caution in women and men of reproductive age due to gonadotoxicity 3

Remember that the goal of treatment is to achieve disease remission or low disease activity while minimizing medication toxicity and preventing long-term organ damage.