What is the recommended treatment for medium vessel vasculitis?

Treatment of Medium Vessel Vasculitis

For medium vessel vasculitis, the recommended first-line treatment is a combination of cyclophosphamide (intravenous or oral) and high-dose glucocorticoids (40-60 mg/day prednisone-equivalent), which should be initiated immediately to induce remission. 1

Initial Treatment Approach

Glucocorticoid Therapy

• Start with high-dose glucocorticoids at 40-60 mg/day prednisone-equivalent
• For severe disease, consider IV methylprednisolone 500-1000 mg/day for 3-5 days before oral therapy
• Taper glucocorticoid dose gradually based on clinical response
• Aim for maintenance dose of 10 mg/day or less during remission 1

Immunosuppressive Therapy

Based on disease severity:

1. Generalized/Severe Disease:

   • Cyclophosphamide (oral or IV) plus glucocorticoids
   • Rituximab plus glucocorticoids (alternative to cyclophosphamide)

2. Non-Organ Threatening Disease:

   • Methotrexate (oral or parenteral) plus glucocorticoids 1

3. Rapidly Progressive Renal Disease:

   • Add plasma exchange to improve renal survival 1

Remission Maintenance Therapy

After achieving remission (typically 3-6 months):

• Switch to maintenance therapy with low-dose glucocorticoids plus one of:

  • Azathioprine (recommended first-line)
  • Leflunomide
  • Methotrexate 1

• Duration: Maintain therapy for at least 18-24 months in patients who remain in complete remission

Management of Refractory or Relapsing Disease

For patients who fail to achieve remission or relapse on standard therapy:

• Consider referral to specialized vasculitis centers
• Alternative options include:
  • Rituximab (particularly effective with 91% remission rate in refractory cases) 1
  • Mycophenolate mofetil
  • Intravenous immunoglobulin (for persistent low disease activity) 1

Special Considerations

Hepatitis-Associated Vasculitis

• For hepatitis C-associated cryoglobulinemic vasculitis: antiviral therapy is recommended 1
• For hepatitis B-associated PAN: combination of antiviral therapy, plasma exchange, and glucocorticoids 1

Infection Prevention

• Trimethoprim/sulfamethoxazole prophylaxis for patients on cyclophosphamide to prevent Pneumocystis jirovecii pneumonia
• Consider mupirocin for chronic nasal Staphylococcus aureus carriers 1

Monitoring and Follow-up

• Regular clinical assessment with structured approach
• Monitor complete blood count, renal function, and urinalysis
• Long-term monitoring for cyclophosphamide-related bladder toxicity (periodic urinalysis)
• Bone protection for patients on long-term glucocorticoids

Important Caveats

• Patients with medium vessel vasculitis should ideally be managed in collaboration with, or at centers of expertise 1
• Tobacco smokers on cyclophosphamide have increased risk of bladder cancer and should be monitored closely
• For patients of childbearing age, fertility preservation counseling is essential before treatment initiation

The treatment approach should be tailored based on disease severity, organ involvement, and patient factors, with the primary goal of achieving remission while minimizing treatment-related complications.