What is the initial treatment for vasculitis?

Initial Treatment for Vasculitis

The initial treatment for vasculitis should be cyclophosphamide (CYC) and corticosteroids, which is the recommended first-line therapy for induction of remission in most forms of vasculitis, particularly ANCA-associated vasculitis. 1

Classification and Treatment Approach

• Vasculitis should be categorized according to vessel size and disease severity to guide appropriate management 2
• Treatment decisions should be based on vasculitis type, disease severity, organ involvement, and patient factors 2, 3

Initial Treatment Regimens

For ANCA-Associated Vasculitis (AAV):

• First-line induction therapy:

  • High-dose glucocorticoids (prednisolone 1 mg/kg/day, maximum 60 mg/day) combined with either: 1, 3
    • Cyclophosphamide (oral 2 mg/kg/day or IV pulsed) OR
    • Rituximab (375 mg/m² weekly for 4 weeks)

• Cyclophosphamide dosing considerations:

  • Oral: 2 mg/kg/day (maximum 200 mg/day) 3
  • IV pulse: 15 mg/kg every 2-3 weeks 4
  • Dose adjustments needed for renal function and age 1

• Rituximab as alternative initial treatment:

  • Recommended for patients without severe disease or when CYC is contraindicated 1
  • Particularly beneficial in younger patients concerned about fertility and those with relapsing disease 2

For Severe Disease:

• Add plasmapheresis for patients with:

  • Rapidly increasing serum creatinine or requiring dialysis 1
  • Diffuse pulmonary hemorrhage 1
  • Overlap syndrome of ANCA vasculitis and anti-GBM GN 1

• For Giant Cell Arteritis:

  • Immediate initiation of high-dose glucocorticoids 2
  • Consider adjunctive immunosuppressive therapy (methotrexate or tocilizumab) as steroid-sparing agents 2

Duration of Initial Treatment

• For ANCA-associated vasculitis:

  • Consider discontinuing CYC therapy after 3 months in patients who remain dialysis-dependent and have no extrarenal manifestations 1
  • Initial high-dose glucocorticoids should be maintained for 1 month, then tapered gradually 3

• For other forms of vasculitis:

  • Polyarteritis nodosa and Churg-Strauss syndrome: optimal treatment duration is 12 months 5
  • Wegener's granulomatosis: more prolonged treatment, at least 18 months 5

Maintenance Therapy

• Maintenance therapy is recommended in patients who have achieved remission 1
• Recommended options include:
  • Azathioprine 1-2 mg/kg/day orally (first-line) 1
  • MMF (up to 1g twice daily) for patients allergic to or intolerant of azathioprine 1
  • Methotrexate (initially 0.3 mg/kg/week, maximum 25 mg/week) for patients intolerant to azathioprine and MMF, but not if GFR is <60 ml/min 1
• Maintenance therapy should continue for at least 18 months in patients who remain in complete remission 1

Supportive Care

• Prophylaxis against Pneumocystis jiroveci with trimethoprim-sulfamethoxazole for all patients on cyclophosphamide 3
• Mesna should be given to patients receiving cyclophosphamide to prevent hemorrhagic cystitis 3
• Bone protection therapy for patients on long-term glucocorticoids 3
• Regular monitoring of blood counts, renal function, and urinalysis 3

Common Pitfalls to Avoid

• Delaying treatment while waiting for biopsy results in rapidly deteriorating patients 2
• Using alternate-day glucocorticoid therapy, which increases risk of relapse 2
• Inadequate initial immunosuppression in severe disease 2
• Changing immunosuppression based on changes in ANCA titer alone 1
• Underestimating the impact of glucocorticoid side effects on patients' quality of life 6

Special Considerations

• Drug-induced vasculitis often resolves with withdrawal of the offending agent alone, without requiring immunosuppressive therapy 7
• Biological agents themselves can induce vasculitis, particularly anti-TNF-α molecules 8
• Patients with AAV should be treated at centers with experience in AAV management 2