What is the initial treatment for vasculitis?

Initial Treatment for Vasculitis

The initial treatment for vasculitis should include high-dose glucocorticoids combined with cyclophosphamide or rituximab, with the specific regimen determined by disease severity and organ involvement. 1

Treatment Based on Disease Severity

Severe/Life-Threatening Vasculitis

• First-line therapy:

  • High-dose glucocorticoids: IV methylprednisolone 500-1000 mg/day for 3-5 days, followed by oral prednisone 1 mg/kg/day (generally up to 80 mg/day) with gradual taper 2, 1
  • PLUS one of the following:
    • Cyclophosphamide: Up to 2 mg/kg/day orally for 3-6 months OR intermittent IV regimen (15 mg/kg IV every 2 weeks for 3 doses, then every 3 weeks) 2
    • Rituximab: 375 mg/m² IV weekly for 4 weeks OR 1000 mg on days 1 and 15 1, 3

• For rapidly progressive renal disease or pulmonary hemorrhage:

  • Add plasmapheresis to the above regimen 2
  • Consider combination of cyclophosphamide and rituximab for severe disease 1

Non-Severe Vasculitis

• High-dose glucocorticoids (as above)
• PLUS methotrexate (up to 25 mg/week) or mycophenolate mofetil (up to 1500 mg twice daily) 2

Special Considerations

Renal Involvement

• For patients with rapidly increasing serum creatinine or requiring dialysis, add plasmapheresis 2
• Consider cyclophosphamide rather than rituximab alone for rapidly progressive renal disease 1
• If patient remains dialysis-dependent after 3 months with no extrarenal manifestations, consider discontinuing cyclophosphamide 2

Pulmonary Hemorrhage

• Add plasmapheresis for patients with diffuse pulmonary hemorrhage 2

Anti-GBM Disease Overlap

• For patients with overlap syndrome of ANCA vasculitis and anti-GBM glomerulonephritis, add plasmapheresis 2

Maintenance Therapy

After achieving remission (typically 3-6 months):

• Recommended maintenance agents:

  • Azathioprine: 1-2 mg/kg/day orally (first choice) 2
  • Methotrexate: 0.3 mg/kg/week (maximum 25 mg/week) if GFR >60 ml/min 2
  • Mycophenolate mofetil: Up to 1500 mg twice daily (for patients intolerant to azathioprine) 2
  • Rituximab: 500 mg IV every 6 months or other maintenance regimen 2, 1

• Duration of maintenance therapy:

  • At least 18 months in patients who remain in complete remission 2
  • No maintenance therapy needed for patients who are dialysis-dependent with no extrarenal manifestations 2

Adjunctive Treatments

• Trimethoprim-sulfamethoxazole: For patients with upper respiratory tract disease 2
• Pneumocystis jirovecii prophylaxis: Recommended for all patients on cyclophosphamide 1
• Bone protection: Osteoporosis prophylaxis for all patients on glucocorticoids 1

Monitoring

• Regular assessment of disease activity
• Complete blood counts, renal function, and urinalysis
• Monitor for treatment toxicity
• Do not change immunosuppression based on ANCA titer changes alone 2

Treatment of Relapses

• For severe relapses (life- or organ-threatening): Treat according to the same guidelines as initial therapy 2
• For non-severe relapses: Consider increasing immunosuppression intensity with agents other than cyclophosphamide, including increasing glucocorticoid dose with or without azathioprine/MMF 2

Treatment-Resistant Disease

For ANCA-associated vasculitis resistant to induction therapy with cyclophosphamide and corticosteroids:

• Add rituximab 2
• Consider IV immunoglobulin or plasmapheresis as alternatives 2

The treatment approach should be aggressive for severe disease to prevent permanent organ damage while balancing the risk of treatment-related complications, particularly infections, which are a major cause of early mortality, especially in elderly patients 4.