What is the treatment for a lupus flare?

Treatment of Lupus Flares

Hydroxychloroquine (HCQ) is the cornerstone of treatment for all lupus flares and should be prescribed for every patient with SLE unless contraindicated, at a dose not exceeding 5 mg/kg real body weight. 1

First-Line Approach to Lupus Flares

Assessment of Flare Severity

1. Determine organ involvement and severity:
   • Mild flare: Skin rashes, arthritis, mild serositis
   • Moderate flare: More extensive skin/joint involvement, moderate serositis
   • Severe flare: Organ-threatening disease (nephritis, neuropsychiatric manifestations, severe cytopenias)

Treatment Algorithm Based on Severity

For Mild to Moderate Flares:

1. Antimalarials:

   • Ensure patient is on hydroxychloroquine (if not, start immediately)
   • Dose: Not exceeding 5 mg/kg real body weight 1
   • Monitor for retinal toxicity: Baseline ophthalmological screening, then after 5 years, and yearly thereafter 1

2. Glucocorticoids (GC):

   • Short course of low-to-moderate dose oral prednisone (or equivalent)
   • Starting dose: 0.5-0.6 mg/kg/day (maximum 40 mg) for mild flares 1
   • Taper according to clinical response following the reduced-dose scheme 1
   • Goal: Minimize to <7.5 mg/day prednisone equivalent as quickly as possible 1

3. NSAIDs:

   • May be used judiciously for short periods in patients at low risk for complications 1
   • Particularly helpful for arthritic symptoms and serositis

For Severe/Organ-Threatening Flares:

1. High-intensity immunosuppressive therapy:

   • IV methylprednisolone pulses (250-1000 mg/day for 1-3 days) 1
   • Follow with oral prednisone at 0.8-1.0 mg/kg/day (maximum 80 mg) 1

2. Immunosuppressive agents:

   • For lupus nephritis: Mycophenolate mofetil (MMF) or cyclophosphamide 1
   • For other severe manifestations: Cyclophosphamide, azathioprine, or mycophenolate 1
   • For refractory disease: Consider rituximab or belimumab 1

3. Maintenance therapy after flare control:

   • Taper glucocorticoids to <7.5 mg/day or withdraw completely if possible 1
   • Continue antimalarials indefinitely 1
   • Maintenance immunosuppression with MMF (750-1000 mg twice daily) or azathioprine for at least 36 months in cases of organ-threatening disease 1

Special Considerations

Lupus Nephritis Flares

• Initial therapy: Glucocorticoids plus either mycophenolate mofetil or cyclophosphamide 1
• Alternative regimens: Consider calcineurin inhibitors (voclosporin, tacrolimus) for patients with preserved kidney function and nephrotic-range proteinuria 1
• Target: At least partial remission (≥50% reduction in proteinuria to subnephrotic levels) by 6-12 months 1
• Complete remission may require longer treatment (up to 24 months) 1

Refractory Disease

• For inadequate response to standard therapy: Consider adding belimumab 1
• For organ-threatening refractory disease: Consider rituximab 1
• Triple immunosuppressive regimen: Belimumab with glucocorticoids and either mycophenolate or reduced-dose cyclophosphamide for high-risk patients 1

Monitoring Response and Preventing Complications

Disease Monitoring

• Clinical signs: Rashes, arthritis, serositis, neurological manifestations
• Laboratory tests: CBC, serum creatinine, proteinuria, urinary sediment, serum C3/C4, anti-dsDNA 1
• For lupus nephritis: Reduction in proteinuria is more important than residual hematuria 1

Prevention of Complications

• Screen for and manage cardiovascular risk factors 1
• Consider low-dose aspirin and/or lipid-lowering agents based on individual risk profile 1
• Bone health: Calcium/vitamin D supplementation, consider bisphosphonates with chronic GC use
• Infection prevention: Consider appropriate vaccinations

Common Pitfalls and Caveats

1. Medication adherence: Poor adherence to hydroxychloroquine is common and associated with increased flare rates. Consider measuring drug levels if available 1

2. Glucocorticoid dependence: Prolonged use of glucocorticoids >7.5 mg/day leads to significant damage accrual. Promptly initiate steroid-sparing agents to minimize GC exposure 1

3. Smoking: Inhibits therapeutic efficacy of antimalarials in cutaneous lupus 2

4. Hydroxychloroquine retinopathy: Risk increases with duration of treatment, especially after 20 years of continuous use. Maintain appropriate dosing and regular screening 1

5. Pregnancy considerations: Hydroxychloroquine should be continued during pregnancy as it decreases lupus activity with a favorable safety profile 1

By following this treatment approach, the goals of therapy should be to achieve remission or low disease activity in all organ systems, prevent damage accrual, and minimize drug side effects, ultimately improving long-term outcomes and quality of life for patients with lupus.