What is the initial treatment approach for patients with vasculitis?

Initial Treatment Approach for Vasculitis

The initial treatment for vasculitis involves high-dose glucocorticoids combined with cyclophosphamide or rituximab for organ/life-threatening disease, with treatment selection based on disease severity and type. 1

Treatment Algorithm Based on Disease Severity

Treatment should be tailored according to disease severity:

Severe/Generalized Vasculitis

• First-line therapy:

  • High-dose glucocorticoids: IV methylprednisolone 500-1000 mg/day for 3 days, followed by oral prednisone 1 mg/kg/day with gradual taper 1
  • PLUS one of the following:
    • Rituximab: 375 mg/m² weekly for 4 weeks 1, 2
    • Cyclophosphamide (alternative to rituximab) 1

• For rapidly progressive severe renal disease:

  • Consider cyclophosphamide rather than rituximab alone, or a combination of both
  • Add plasma exchange to the treatment regimen 1

Non-Severe Vasculitis

• Methotrexate + Glucocorticoids 1

Refractory/Relapsing Disease

• Tocilizumab or cyclophosphamide 1

Special Considerations

ANCA-Associated Vasculitis (GPA/MPA)

• Rituximab (375 mg/m² weekly for 4 weeks) + glucocorticoids is the first choice 1, 2
• Cyclophosphamide + glucocorticoids is an acceptable alternative 1
• For patients at high risk of glucocorticoid toxicity, avacopan (30 mg twice daily) may be considered as an alternative to glucocorticoids 1

Important Precautions

• Cyclophosphamide contraindications:

  • Pregnancy (alternative treatments should be considered) 1
  • Requires fertility preservation counseling before initiation, especially for patients of childbearing age 1

• Rituximab contraindications:

  • Active infections
  • Certain immunodeficiencies 1, 2

Adjunctive Treatments

• Pneumocystis jirovecii prophylaxis: Trimethoprim/sulfamethoxazole (800/160 mg on alternate days or 400/80 mg daily) for all patients on cyclophosphamide 1

• Bone protection: Osteoporosis prophylaxis for all patients on glucocorticoids 1

• Blood pressure management:

  • Target BP <140/90 mmHg (or <130/80 mmHg for patients with diabetes or chronic kidney disease) 1
  • Calcium channel blockers (e.g., amlodipine) are preferred first-line agents for patients with renal involvement 1

Monitoring

Regular monitoring is essential for:

• Disease activity
• Treatment toxicity
• Complete blood counts
• Renal function
• Urinalysis 1

Maintenance Therapy

After remission is achieved, maintenance therapy typically involves:

• Rituximab or azathioprine with low-dose glucocorticoids
• Duration of at least 18 months to 4 years 1

The treatment approach should be aggressive from the outset for severe disease to prevent permanent organ damage or death, while carefully balancing the risk of treatment-related adverse effects.