What is the treatment for vasculitis?

Treatment of Vasculitis

Vasculitis requires immediate combination therapy with high-dose glucocorticoids (prednisolone 1 mg/kg/day, maximum 60 mg/day) plus either rituximab or cyclophosphamide, with treatment selection determined by vasculitis type, disease severity, and organ involvement. 1

Initial Assessment and Treatment Urgency

• Do not delay treatment while waiting for biopsy results in rapidly deteriorating patients with positive ANCA serology and compatible clinical presentation—start immunosuppression immediately. 1, 2
• Categorize disease by vessel size (small, medium, or large vessel) and severity (localized, early systemic, generalized, severe, or refractory) to guide treatment intensity. 1, 3
• Untreated systemic vasculitis carries 40-46% five-year mortality in patients with poor prognostic factors—delaying proven immunosuppressive therapy risks permanent organ damage or death. 3

ANCA-Associated Vasculitis (Wegener's Granulomatosis, Microscopic Polyangiitis)

Induction Therapy

For generalized or severe ANCA-associated vasculitis, initiate cyclophosphamide (2 mg/kg/day oral, maximum 200 mg/day, OR intravenous pulses) PLUS prednisolone (1 mg/kg/day, maximum 60 mg/day). 1, 3

• Rituximab 375 mg/m² weekly for 4 weeks is equally effective as cyclophosphamide and achieves 64% complete remission at 6 months. 1, 4
• Prefer rituximab over cyclophosphamide for:
  • Younger patients with fertility concerns 1, 3
  • Relapsing disease (67% vs 42% remission rate compared to cyclophosphamide) 1
• Prefer cyclophosphamide for:
  • Severe renal disease with markedly reduced or rapidly declining renal function 1
  • Diffuse alveolar hemorrhage with hypoxemia 1
• For severe disease with pulmonary hemorrhage or rapidly progressive renal failure requiring dialysis, consider combination of rituximab plus cyclophosphamide or add plasma exchange. 1

Maintenance Therapy

Once remission is achieved, transition to azathioprine 1-2 mg/kg/day (first-line choice with strongest evidence) PLUS low-dose glucocorticoids (5-7.5 mg/day). 1, 2

• Alternative maintenance options include rituximab, mycophenolate mofetil, or leflunomide. 1
• Continue maintenance therapy for minimum 18-24 months in complete remission; extending to 4 years reduces relapse risk. 1, 2
• Do not use methotrexate if GFR <60 mL/min per 1.73 m². 1

Refractory Disease

• For treatment failure or relapse, rituximab achieves 91% remission rate in refractory ANCA-associated vasculitis. 1, 2
• Intravenous immunoglobulin (IVIG) 2 g/kg can provide short-term control, but measure serum immunoglobulin levels first due to anaphylaxis risk in IgA deficiency. 1, 3

Large Vessel Vasculitis (Giant Cell Arteritis, Takayasu Arteritis)

Giant Cell Arteritis

Initiate high-dose glucocorticoids immediately (prednisolone 1 mg/kg/day, typically 40-60 mg/day) to reduce recurrent stroke risk. 1, 3

• Maintain high-dose glucocorticoids for minimum 1 month before gradual tapering. 1
• Add methotrexate 10-15 mg/week as steroid-sparing agent to reduce relapse rate and cumulative glucocorticoid dose. 3
• Consider tocilizumab as alternative steroid-sparing agent. 1
• Prescribe low-dose aspirin 75-150 mg/day for all patients to prevent cardiovascular and cerebrovascular events. 3

Takayasu Arteritis

All patients should receive non-biological glucocorticoid-sparing agents (methotrexate 20-25 mg/week preferred) in combination with glucocorticoids from the start. 1, 3

• Monitor disease activity with clinical assessment, inflammatory markers (ESR, CRP), and consider periodic MRI or PET imaging. 3

Cutaneous Vasculitis

Isolated Cutaneous Disease (Non-Organ Threatening)

• For mild, self-limited cutaneous vasculitis: leg elevation, avoid prolonged standing, NSAIDs. 5
• For mild recurrent or persistent disease: colchicine or dapsone as first-choice agents. 5

Severe Cutaneous or Systemic Disease with Cutaneous Involvement

For gangrene or severe organ-threatening cutaneous vasculitis, use immediate high-dose glucocorticoids (prednisone 1 mg/kg/day) combined with cyclophosphamide or rituximab. 2

Essential Supportive Measures

• Provide Pneumocystis jiroveci prophylaxis (trimethoprim-sulfamethoxazole) for all patients receiving cyclophosphamide. 1, 3
• Prescribe bone protection with calcium, vitamin D, and bisphosphonates for all patients on glucocorticoids. 2
• Use Mesna as uroprotective agent with cyclophosphamide to reduce bladder toxicity risk (though it does not eliminate risk entirely). 3

Monitoring Requirements

• Perform structured clinical assessment, urinalysis, and basic laboratory tests (CBC, ESR, CRP, kidney function) at every visit. 1, 3
• Investigate persistent unexplained hematuria in all patients with prior cyclophosphamide exposure due to bladder cancer risk—tobacco smokers develop bladder cancer at lower doses and earlier than non-smokers. 1
• ANCA persistence, increase in levels, or change from negative to positive may predict future relapse and should inform treatment decisions. 1

Critical Pitfalls to Avoid

• Never delay treatment in rapidly deteriorating patients while waiting for biopsy confirmation. 1, 2
• Never use alternate-day glucocorticoid therapy—this increases relapse risk. 1
• Avoid inadequate initial immunosuppression in severe disease. 1
• Refer patients to centers with experience in vasculitis management, particularly for ANCA-associated vasculitis. 1, 3