Treatment Modalities for Systemic Lupus Erythematosus (SLE)
Hydroxychloroquine should be prescribed to all patients with SLE unless contraindicated, as it forms the cornerstone of treatment by reducing disease activity, preventing flares, and improving survival. 1
First-Line Treatments
- Hydroxychloroquine: FDA-approved for SLE 2, should be used in all patients regardless of disease severity or organ involvement unless contraindicated
- NSAIDs: For mild symptoms, particularly musculoskeletal manifestations
- Glucocorticoids: Used for disease flares and active disease
- Low-dose prednisone (≤7.5 mg/day) for mild-moderate disease activity
- High-dose or pulse methylprednisolone for severe manifestations
Treatment Based on Disease Severity
Mild SLE (without major organ involvement)
- Hydroxychloroquine
- NSAIDs for symptomatic relief
- Low-dose glucocorticoids if needed
- Sun protection and lifestyle modifications
Moderate to Severe SLE (with organ involvement)
- Hydroxychloroquine (continue as background therapy)
- Immunosuppressive agents based on specific organ involvement:
- Azathioprine (1-2 mg/kg/day): Useful as maintenance therapy 1
- Mycophenolate mofetil: Effective for lupus nephritis and as alternative to azathioprine 1
- Cyclophosphamide: Reserved for severe manifestations, especially lupus nephritis and neuropsychiatric SLE 1
- Belimumab: FDA-approved for active SLE and lupus nephritis in patients with positive autoantibodies 3, 1
Organ-Specific Treatment Approaches
Lupus Nephritis
Treatment based on nephritis class:
- Class III/IV: Methylprednisolone pulses followed by oral prednisone with mycophenolate mofetil or cyclophosphamide 1
- Class V: Prednisone with mycophenolate mofetil 1
- High-risk: High-dose intravenous cyclophosphamide 1
- Belimumab: Can be added for patients with active lupus nephritis 3, 4
- Voclosporin: FDA-approved specifically for lupus nephritis 4
Neuropsychiatric SLE (NPSLE)
- Diagnostic evaluation should be similar to non-SLE patients with same neuropsychiatric manifestations 5
- Treatment options:
Antiphospholipid Syndrome in SLE
- Venous thrombosis: Anticoagulation with warfarin (target INR 2.0-3.0) 5, 1
- Arterial or recurrent thrombosis: High-intensity anticoagulation (target INR 3.0-4.0) 5, 1
- Primary prevention: Low-dose aspirin in SLE patients with antiphospholipid antibodies 1
Newer Biologic Therapies
Belimumab: First FDA-approved biologic for SLE, targets B-cell activating factor (BAFF/BLyS) 3, 6
Anifrolumab: Recently FDA-approved for active SLE 4
Special Considerations
Pregnancy
- Compatible medications: Hydroxychloroquine, azathioprine, low-dose aspirin 1
- Contraindicated medications: Mycophenolate mofetil, cyclophosphamide, methotrexate 1
Preventive Measures
- Sun protection
- Vaccination (inactivated vaccines recommended; live vaccines avoided during immunosuppression) 1
- Cardiovascular risk factor control
- Osteoporosis prevention with calcium, vitamin D, and bisphosphonates
- Lifestyle modifications: regular exercise, smoking cessation, weight control, stress management 1
Monitoring
- Regular assessment of disease activity using validated indices (SLEDAI, BILAG, SLE-DAS) 1
- Monitoring of anti-dsDNA antibodies and complement levels at follow-up visits 1
- Evaluation of accumulated damage using SLICC/ACR index 1
Common Pitfalls and Caveats
- Do not initiate treatment with immunosuppressants based solely on serological values without clinical activity 1
- Avoid live vaccines in patients receiving immunosuppressants or glucocorticoids at doses >20 mg/day 1
- Consider gonadotoxicity when using cyclophosphamide in women and men of fertile age 1
- Hydroxychloroquine should not be discontinued during pregnancy as it reduces flares and improves outcomes 1
- Regular ophthalmologic monitoring is required for patients on hydroxychloroquine to detect early retinopathy