What is the recommended treatment for vasculitis?

Treatment Recommendations for Vasculitis

The recommended treatment for vasculitis depends on the specific type and severity, with high-dose glucocorticoids as the initial therapy for most forms, combined with appropriate immunosuppressive agents based on disease classification and organ involvement. 1, 2

Classification and Initial Assessment

• Vasculitis should be categorized by vessel size (large, medium, small) and disease severity to guide appropriate management 2
• ANCA-associated vasculitis (AAV) should be further classified as: localized, early systemic, generalized, severe, or refractory disease 1, 2
• Treatment should not be delayed while waiting for biopsy results in rapidly deteriorating patients with suspected vasculitis 1, 2

Treatment by Vasculitis Type

Large Vessel Vasculitis (Giant Cell Arteritis and Takayasu Arteritis)

• Initiate high-dose glucocorticoid therapy (40-60 mg/day prednisone-equivalent) immediately for induction of remission 1, 2
• Maintain high-dose glucocorticoids for one month before gradual tapering; avoid alternate-day therapy which increases relapse risk 1, 2
• Consider adjunctive immunosuppressive therapy in selected patients with GCA who have:
  • Refractory or relapsing disease
  • Increased risk for glucocorticoid-related adverse events
  • Complications 1, 2
• For GCA, tocilizumab is recommended as the preferred adjunctive therapy, with methotrexate as an alternative 1, 2, 3
• For Takayasu arteritis, all patients should receive non-biological glucocorticoid-sparing agents in combination with glucocorticoids 1, 2

ANCA-Associated Vasculitis (Granulomatosis with Polyangiitis, Microscopic Polyangiitis, Eosinophilic Granulomatosis with Polyangiitis)

• For induction therapy in generalized disease:

  • Combination of cyclophosphamide (oral or IV) and glucocorticoids is recommended 1, 2
  • Rituximab is an effective alternative to cyclophosphamide, particularly in younger patients concerned about fertility and those with relapsing disease 1, 2, 4
  • For severe disease with pulmonary hemorrhage or rapidly progressive renal disease, consider combination of rituximab and cyclophosphamide 1, 2

• For non-organ threatening or non-life threatening disease:

  • Methotrexate (20-25 mg/week) combined with glucocorticoids is recommended as a less toxic alternative to cyclophosphamide 1, 2
  • Mycophenolate mofetil can be considered in patients who cannot tolerate methotrexate 1, 2

• For maintenance therapy after remission:

  • Either rituximab or azathioprine with low-dose glucocorticoids is recommended 1, 2
  • Optimal duration is between 18 months and 4 years after induction of remission 1

• For severe renal disease:

  • Plasma exchange should be considered for patients with serum creatinine >3.4 mg/dl, requiring dialysis, or with rapidly increasing creatinine 1
  • Also consider plasma exchange for patients with diffuse alveolar hemorrhage who have hypoxemia 1

Dosing Guidelines

• Glucocorticoids:

  • Initial dose: 1 mg/kg/day (maximum 60 mg/day) 1, 2
  • Maintain high dose for 1 month, then taper gradually 1
  • Do not reduce to less than 15 mg/day for the first 3 months 1
  • Taper to maintenance dose of 10 mg/day or less during remission 1

• Cyclophosphamide:

  • Oral: 2 mg/kg/day (maximum 200 mg/day) for 3-6 months 1
  • IV: 15 mg/kg at weeks 0,2,4,7,10,13 1
  • Dose reduction for age: 60+ years (1.5 mg/kg/day oral or 12.5 mg/kg IV), 70+ years (1.0 mg/kg/day oral or 10 mg/kg IV) 1
  • Reduce dose for impaired renal function (GFR <30 ml/min/1.73 m²) 1

• Rituximab:

  • Induction: 375 mg/m² weekly for 4 weeks 1, 4
  • Maintenance: Either 500 mg every 6 months or 1000 mg every 4 months 1

Supportive Care and Monitoring

• Provide prophylaxis against Pneumocystis jiroveci with trimethoprim/sulfamethoxazole in all patients on cyclophosphamide 1, 2
• Implement bone protection therapy for patients on glucocorticoids according to local guidelines 1, 2
• For patients receiving cyclophosphamide, administer Mesna to prevent hemorrhagic cystitis 1
• Monitor complete blood count regularly; adjust or discontinue immunosuppressants if acute leucopenia or progressive decline occurs 1, 2
• Monitor renal function and urinalysis to assess for disease activity and medication toxicity 1, 2
• Follow ANCA levels as persistence or increase may predict future relapse 1, 2

Common Pitfalls to Avoid

• Delaying treatment while waiting for biopsy results in rapidly deteriorating patients 1, 2
• Using alternate-day glucocorticoid therapy, which increases risk of relapse 1, 2
• Inadequate initial immunosuppression in severe disease 1, 2
• Failing to provide prophylaxis against opportunistic infections and osteoporosis 1, 2
• Overlooking the significant burden of glucocorticoid-related side effects, which greatly impact patients' quality of life 5
• Not considering steroid-sparing agents early enough in treatment course, especially in patients at high risk for steroid complications 1, 2