What are the first-line medications for lupus?

First-Line Medications for Lupus

Hydroxychloroquine is the first-line medication for all patients with systemic lupus erythematosus (SLE), regardless of disease severity or organ involvement. 1

Treatment Algorithm Based on Disease Severity

All Patients with SLE

• Hydroxychloroquine should be prescribed at a dose not exceeding 5 mg/kg real body weight 1
• Regular ophthalmological screening is required: at baseline, after 5 years, and yearly thereafter to monitor for retinal toxicity 1
• Hydroxychloroquine has demonstrated high-quality evidence for improving outcomes by reducing renal flares and limiting accrual of renal and cardiovascular damage 1
• Effectiveness ranges from 50%-97% in cutaneous lupus erythematosus (CLE) with dosages up to 400 mg/day 2

Disease-Specific Treatment Approach

Mild Disease

• Hydroxychloroquine alone is typically sufficient 1
• Low-dose glucocorticoids (prednisone <7.5 mg/day) may be added for symptom control 1

Moderate to Severe Disease

• Continue hydroxychloroquine as the foundation therapy 1
• Add glucocorticoids at doses dependent on disease severity:
  • Initial treatment may include pulses of intravenous methylprednisolone (250-1000 mg/day for 1-3 days) 1
  • Oral prednisone should be minimized to less than 7.5 mg/day for chronic maintenance 1

Organ-Threatening Disease

• Hydroxychloroquine remains the foundation therapy 1
• Immunosuppressive agents should be included in initial therapy:
  • For lupus nephritis: mycophenolate mofetil or low-dose intravenous cyclophosphamide are recommended as initial treatments 1
• For refractory cases, consider rituximab (though evidence quality is rated as low) 1

Monitoring and Follow-up

• Disease activity monitoring schedule:
  • Every 2-4 weeks for the first 2-4 months after diagnosis or flare 1
  • Assessment should include: body weight, blood pressure, serum creatinine, eGFR, serum albumin, proteinuria, urinary sediment, complement levels, and anti-dsDNA antibody levels 1
  • Long-term follow-up should continue at least every 3-6 months 1

Special Considerations

• Medication adherence is critical to preventing disease flares 1
• For patients with inadequate response to standard therapy, belimumab may be considered as add-on treatment (moderate strength of evidence) 1, 3
• Triple immunosuppressive regimens (belimumab with glucocorticoids and either mycophenolate or reduced-dose cyclophosphamide) may benefit patients with repeated kidney flares 1

Important Practical Tips

• When reducing hydroxychloroquine in well-controlled patients, decrease by no more than approximately 600 mg per week 4
• Consider timing of hydroxychloroquine administration:
  • Morning administration for patients with insomnia 4
  • Evening administration for patients with dyspepsia 4
  • Separate intake from showering in patients with aquagenic pruritus 4
• Retinopathy is an exceedingly rare adverse effect with appropriate dosing and monitoring 2