What is the treatment for different types of vasculitis?

Treatment of Different Types of Vasculitis

The treatment of vasculitis must be tailored according to the specific type and severity of disease, with cyclophosphamide plus glucocorticoids being the cornerstone therapy for severe, organ-threatening forms of small and medium vessel vasculitis. 1

Classification and Severity Assessment

Vasculitis treatment depends primarily on:

1. Type of vasculitis (ANCA-associated vs. non-ANCA-associated)
2. Disease severity (organ-threatening vs. non-organ-threatening)
3. Disease stage (induction vs. maintenance therapy)

The European Vasculitis Study (EUVAS) categorization for ANCA-associated vasculitis provides a useful framework:

Category	Definition	Treatment Approach
Localized	Upper/lower respiratory tract disease only	Less aggressive therapy
Early systemic	Any, without organ-threatening disease	Methotrexate + glucocorticoids
Generalized	Organ-threatening disease, creatinine <500 μmol/L	Cyclophosphamide/rituximab + glucocorticoids
Severe	Renal/vital organ failure, creatinine >500 μmol/L	Cyclophosphamide/rituximab + glucocorticoids + plasma exchange
Refractory	Progressive disease despite therapy	Alternative immunosuppressants

Treatment by Vasculitis Type

1. ANCA-Associated Vasculitis (AAV)

(Granulomatosis with Polyangiitis [GPA], Microscopic Polyangiitis [MPA], Eosinophilic Granulomatosis with Polyangiitis [EGPA])

Remission Induction for Severe/Generalized Disease:

• First-line therapy: Cyclophosphamide (oral or IV) plus high-dose glucocorticoids 1

  • Cyclophosphamide: 2 mg/kg/day orally (max 200 mg/day) or IV pulse therapy
  • Prednisolone: 1 mg/kg/day (max 60 mg/day)
  • Duration: 3-6 months

• Alternative first-line: Rituximab plus glucocorticoids 2

  • Rituximab: 375 mg/m² IV weekly for 4 weeks
  • Equally effective as cyclophosphamide for remission induction

• Adjunctive therapy:

  • Plasma exchange for rapidly progressive renal disease 1
  • Pneumocystis jiroveci prophylaxis with trimethoprim/sulfamethoxazole 1
  • Mesna for patients receiving cyclophosphamide to prevent bladder toxicity 1

Remission Induction for Non-Severe Disease:

• First-line: Methotrexate plus glucocorticoids 1
  • Less toxic alternative to cyclophosphamide
  • For patients without organ-threatening manifestations

For EGPA Specifically:

• Severe disease: Cyclophosphamide or rituximab + glucocorticoids 1
• Non-severe disease: Mepolizumab + glucocorticoids (first choice) or methotrexate/azathioprine/mycophenolate mofetil + glucocorticoids 1

Remission Maintenance:

• First-line: Azathioprine + low-dose glucocorticoids 1
• Alternatives: Methotrexate or leflunomide + low-dose glucocorticoids 1
• Duration: At least 18 months, longer for GPA 3

2. Polyarteritis Nodosa (PAN)

• With poor prognostic factors: Cyclophosphamide + glucocorticoids 1, 3
• Without poor prognostic factors: Glucocorticoids alone 3
• Hepatitis B-associated PAN: Antiviral therapy + immunosuppression 1
• Duration: Typically 12 months 3

3. Cryoglobulinemic Vasculitis

• Hepatitis C-associated: Antiviral therapy 1
• Non-viral (essential): Immunosuppressive therapy similar to other small vessel vasculitides 1
• Severe cases: Rituximab has shown benefit 4

4. Cutaneous Vasculitis

• Mild disease: NSAIDs, leg elevation, avoiding triggers 5, 4
• Persistent/recurrent disease: Colchicine or dapsone 5, 4
• Severe cutaneous disease: Systemic glucocorticoids + immunosuppressants 5, 4

Monitoring During Treatment

1. Regular laboratory monitoring:

   • Complete blood count with differential
   • Renal function tests
   • Urinalysis
   • Inflammatory markers (ESR, CRP)
   • ANCA levels (may predict relapse in some patients) 6

2. Monitor for treatment complications:

   • Lymphopenia: Associated with increased infection risk; monitor lymphocyte counts regularly 7
   • Leukopenia: May require dose adjustment or discontinuation of immunosuppressants 1
   • Declining renal function: May necessitate dose adjustment 1
   • Hyperglycemia: Monitor blood glucose while on glucocorticoids 1

Management of Refractory Disease

For patients who fail to respond to standard therapy:

1. Consider alternative agents:

   • Mycophenolate mofetil
   • Intravenous immunoglobulin (IVIG)
   • Rituximab (if not used initially)
   • Anti-TNF agents (e.g., infliximab) 1, 5, 4

2. Refer to specialized centers for enrollment in clinical trials 1

Important Considerations and Pitfalls

• Don't delay treatment in rapidly progressive disease while waiting for biopsy results 6
• Beware of infection risk with immunosuppression, especially with severe lymphopenia 7
• Adjust therapy based on changing disease category - patients may progress from limited to severe disease 1
• Consider prophylaxis against Pneumocystis jiroveci in all patients on cyclophosphamide 1
• Monitor for drug toxicity according to standard protocols 1

By following this structured approach based on disease type and severity, outcomes for patients with vasculitis have dramatically improved from what was once a frequently fatal disease to a manageable chronic condition.