What is the treatment for vasculitis?

Treatment for Vasculitis

The treatment for vasculitis requires a combination of cyclophosphamide (intravenous or oral) and glucocorticoids for remission induction in generalized primary small and medium vessel vasculitis. 1 Treatment should be tailored according to disease severity, type of vasculitis, and organ involvement.

Classification and Assessment

Before initiating treatment, patients with vasculitis should be:

1. Managed in collaboration with, or at centers of expertise 1
2. Categorized according to disease severity to guide treatment decisions 1

ANCA-Associated Vasculitis Disease Categories:

• Localized: Upper/lower respiratory tract disease without systemic involvement
• Early systemic: Any disease without organ-threatening or life-threatening manifestations
• Generalized: Renal or other organ-threatening disease, serum creatinine <500 μmol/L
• Severe: Renal or other vital organ failure, serum creatinine >500 μmol/L
• Refractory: Progressive disease unresponsive to glucocorticoids and cyclophosphamide

Treatment Approach Based on Disease Severity

1. Generalized/Severe Disease

Remission Induction:

• First-line: Cyclophosphamide (oral or IV) plus high-dose glucocorticoids 1
  • Oral cyclophosphamide: 2 mg/kg/day (max 200 mg/day)
  • Prednisolone: 1 mg/kg/day (max 60 mg/day)
  • Initial high-dose steroids should be maintained for 1 month, not reduced to less than 15 mg/day for first 3 months

Additional Measures:

• Patients receiving cyclophosphamide should receive Mesna to prevent bladder toxicity 1
• Pneumocystis jiroveci prophylaxis with trimethoprim/sulfamethoxazole (800/160 mg on alternate days or 400/80 mg daily) 1

For Severe Renal Disease:

• Plasma exchange is recommended for patients with rapidly progressive severe renal disease 1

2. Non-Severe/Early Systemic Disease

Remission Induction:

• First-line: Methotrexate (oral or parenteral) plus glucocorticoids 1
  • Start methotrexate at 15 mg/week and escalate to 20-25 mg/week over 1-2 months
  • Consider folic acid supplementation

3. For Eosinophilic Granulomatosis with Polyangiitis (EGPA)

• Active severe EGPA: Cyclophosphamide or rituximab with glucocorticoids 1
• Active non-severe EGPA: Mepolizumab with glucocorticoids is preferred over methotrexate, azathioprine, or mycophenolate mofetil 1

4. For Giant Cell Arteritis

• Immediate treatment: High-dose glucocorticoids 1
• Adjunctive therapy: Methotrexate or tocilizumab with steroids 1
• Avoid: Infliximab (associated with recurrent symptoms) 1

Remission Maintenance

After achieving remission, maintenance therapy should include:

• Low-dose glucocorticoids (target ≤10 mg/day)
• Plus one of the following:
  • Azathioprine (strongest evidence)
  • Leflunomide
  • Methotrexate 1

Treatment of Refractory Disease

For patients who fail to achieve remission on standard therapy:

• Consider rituximab (particularly effective in ANCA-associated vasculitis) 1, 2
• Other options include mycophenolate mofetil, intravenous immunoglobulin, or other biologics 1

Monitoring and Follow-up

• Regular monitoring of complete blood count, renal function, and urinalysis 1
• Dose adjustments or discontinuation of immunosuppressants may be necessary with leucopenia or declining renal function 1
• Regular blood sugar monitoring while on glucocorticoid therapy 1
• Long-term monitoring for bladder cancer in patients exposed to cyclophosphamide 1

Special Considerations

• Cryoglobulinemic vasculitis: Requires immunosuppressive therapy; if hepatitis C-associated, antiviral therapy is recommended 1
• Hepatitis B-associated PAN: Combination of antiviral therapy, plasma exchange, and glucocorticoids 1
• Cutaneous vasculitis: For mild disease, consider colchicine or dapsone; for severe disease, systemic immunosuppression may be required 3, 4

Common Pitfalls to Avoid

• Delaying treatment in suspected severe vasculitis
• Inadequate Pneumocystis prophylaxis during immunosuppression
• Failing to monitor for drug toxicity (especially cyclophosphamide-induced leucopenia and bladder toxicity)
• Tapering glucocorticoids too rapidly, which may lead to disease flares
• Not considering disease severity when selecting treatment regimen

Remember that early diagnosis and appropriate treatment significantly improve outcomes in patients with vasculitis, particularly in severe forms like Wegener's granulomatosis and polyarteritis nodosa 5.