What is the recommended treatment for vasculitis?

Treatment of Vasculitis

The recommended first-line treatment for vasculitis is high-dose glucocorticoids combined with either rituximab or cyclophosphamide, with rituximab preferred due to its comparable efficacy and better safety profile. 1

Classification-Based Treatment Approach

Treatment for vasculitis should be tailored according to the type of vasculitis and disease severity:

Large Vessel Vasculitis (GCA, Takayasu Arteritis)

• Initial therapy: High-dose glucocorticoids (40-60 mg/day prednisone-equivalent) should be started immediately 2
• Dosing: 1 mg/kg/day (maximum 60 mg/day) maintained for one month, then tapered gradually 2
• Adjunctive therapy:
  • For GCA: Tocilizumab recommended for refractory/relapsing disease or patients at high risk for glucocorticoid-related adverse events 2
  • Methotrexate may be used as an alternative 2
  • For Takayasu arteritis: Non-biological immunosuppressants should be given with glucocorticoids in all patients 2

Small and Medium Vessel Vasculitis (ANCA-Associated)

Disease Categorization

Treatment should be based on disease severity 2:

• Localized: Upper/lower respiratory tract disease without systemic involvement
• Early systemic: Without organ-threatening manifestations
• Generalized: Renal or other organ-threatening disease (creatinine <500 μmol/L)
• Severe: Renal or vital organ failure (creatinine >500 μmol/L)
• Refractory: Progressive disease unresponsive to standard therapy

Remission Induction

1. For generalized/severe disease:

   • First choice: Rituximab (375 mg/m² weekly for 4 weeks) + glucocorticoids 2, 1, 3
   • Alternative: Cyclophosphamide + glucocorticoids 2, 1
     • Oral: 2 mg/kg/day (max 200 mg/day) for 3-6 months
     • IV pulse: 15 mg/kg at weeks 0,2,4,7,10,13 (with dose adjustments for age and renal function)

2. For non-severe disease:

   • Methotrexate (20-25 mg/week) + glucocorticoids 2
   • Mycophenolate mofetil (2000-3000 mg/day) for patients who cannot take methotrexate 2

3. Glucocorticoid regimen:

   • Initial dose: 1 mg/kg/day (max 60 mg/day) 2
   • For severe disease: Consider IV methylprednisolone pulse (500-1000 mg/day for 3 days) 1
   • Maintain high dose for 1 month, then taper gradually 2
   • Do not use alternate-day therapy (increases relapse risk) 2

4. Special situations:

   • For rapidly progressive severe renal disease: Add plasma exchange 2
   • For patients at high risk of glucocorticoid toxicity: Consider avacopan (30 mg twice daily) as alternative to glucocorticoids 2

Remission Maintenance

1. Recommended agents:

   • Rituximab (500 mg every 6 months) OR
   • Azathioprine (1.5-2 mg/kg/day) with low-dose glucocorticoids 2

2. Duration of maintenance therapy:

   • 18 months to 4 years after induction of remission 2
   • For high-risk patients (PR3-ANCA positive), consider longer treatment 2

Important Adjunctive Measures

• Pneumocystis jirovecii prophylaxis: Trimethoprim/sulfamethoxazole (800/160 mg on alternate days or 400/80 mg daily) for all patients on cyclophosphamide 2, 1
• Bone protection: All patients should receive osteoporosis prophylaxis while on glucocorticoids 2, 1
• For cyclophosphamide therapy:
  • Use Mesna to prevent bladder toxicity 2, 1
  • Regular monitoring of blood counts and renal function 2
• Antiplatelet/anticoagulant therapy: Not routinely recommended unless indicated for other reasons 2

Common Pitfalls and Caveats

1. Delayed treatment: Do not delay treatment of suspected GCA while awaiting biopsy results; treatment should be started immediately on strong clinical suspicion 2

2. Inadequate glucocorticoid tapering: Too rapid tapering increases relapse risk; avoid alternate-day therapy 2

3. Undertreatment of Takayasu arteritis: All patients should receive immunosuppressive agents in combination with glucocorticoids 2

4. Overlooking prophylaxis: Failure to provide Pneumocystis prophylaxis and bone protection can lead to serious complications 2, 1

5. Inadequate monitoring: Regular assessment of disease activity and treatment toxicity is essential 2

6. Inappropriate treatment duration: Premature discontinuation of therapy increases relapse risk; maintenance therapy should typically continue for at least 18 months 2, 1

By following this evidence-based approach and avoiding common pitfalls, the morbidity and mortality associated with vasculitis can be significantly reduced, improving patient outcomes and quality of life.