What is the treatment for vasculitis?

Treatment for Vasculitis

Vasculitis treatment requires immediate combination therapy with high-dose glucocorticoids plus either rituximab or cyclophosphamide, with the specific regimen determined by vasculitis type, disease severity, and organ involvement. 1, 2

Initial Assessment and Disease Classification

Before initiating treatment, vasculitis must be categorized by:

• Vessel size involvement (large, medium, or small vessel) to guide therapeutic approach 2, 3
• Disease severity using validated tools such as Birmingham Vasculitis Activity Score (BVAS), categorizing patients as localized, early systemic, generalized, severe, or refractory 2, 3
• Organ involvement, particularly renal function (GFR) and pulmonary status, as these determine treatment intensity 1, 3

Critical pitfall to avoid: Do not delay treatment while waiting for biopsy results in rapidly deteriorating patients with positive ANCA serology, as this increases morbidity and mortality risk 2

Treatment by Vasculitis Type

ANCA-Associated Vasculitis (Small Vessel)

Induction Therapy for Severe/Generalized Disease:

• Glucocorticoids: Prednisolone 1 mg/kg/day (maximum 60 mg/day) initiated immediately, maintained for 1 month before tapering 1, 2, 3
• Plus either:
  • Rituximab: 375 mg/m² weekly for 4 weeks 1, 2, 4
  • OR Cyclophosphamide: 2 mg/kg/day orally (maximum 200 mg/day) or IV pulse dosing 1, 2, 3

When to choose rituximab over cyclophosphamide 1, 2:

• Relapsing disease (rituximab superior: 67% vs 42% remission rate) 4
• Younger patients concerned about fertility
• Previous cyclophosphamide exposure

When to choose cyclophosphamide 1, 2:

• Severe renal disease with markedly reduced or rapidly declining GFR
• Diffuse alveolar hemorrhage with hypoxemia
• Consider combination rituximab + cyclophosphamide for most severe presentations 2

Adjunctive therapy for severe disease 1:

• Plasma exchange for patients with dialysis requirement, rapidly progressive renal failure, or diffuse alveolar hemorrhage with hypoxemia 1, 5
• IV methylprednisolone 1,000 mg daily for 1-3 days prior to initial infusion 6

Maintenance Therapy (After Achieving Remission)

Three evidence-based options 1:

1. Rituximab (preferred for relapsing disease):

   • MAINRITSAN scheme: 500 mg at remission, then 500 mg at months 6,12, and 18 1, 3
   • OR RITAZAREM scheme: 1,000 mg at remission, then at months 4,8,12, and 16 1

2. Azathioprine:

   • 1.5-2 mg/kg/day for 18-24 months 1, 3
   • Then decrease to 1 mg/kg/day until 4 years after diagnosis 1
   • Taper by 25 mg every 3 months after 4 years 1

3. Mycophenolate mofetil:

   • 2,000 mg/day (divided doses) for 2 years 1

Glucocorticoid maintenance: Continue 5-7.5 mg/day for 2 years, then reduce by 1 mg every 2 months 1, 3

Critical contraindication: Methotrexate should NOT be used in patients with GFR <60 ml/min per 1.73 m² 1

Large Vessel Vasculitis (Giant Cell Arteritis, Takayasu Arteritis)

Initial treatment 2:

• High-dose glucocorticoids 1 mg/kg/day (maximum 60 mg/day) initiated immediately
• Maintain high dose for 1 month before gradual tapering to 15 mg/day or less over first 3 months

Steroid-sparing agents 2:

• Giant Cell Arteritis: Consider methotrexate or tocilizumab as adjunctive therapy
• Takayasu Arteritis: ALL patients should receive non-biological glucocorticoid-sparing agents in combination with glucocorticoids

Critical pitfall: Never use alternate-day glucocorticoid therapy, as this significantly increases relapse risk 2, 7

Management of Refractory or Relapsing Disease

Relapsing disease 1, 3:

• Reinduction therapy, preferably with rituximab (even if cyclophosphamide was used initially)

Refractory disease 1:

• Increase glucocorticoids (IV or oral)
• Add rituximab if cyclophosphamide was used previously, or vice versa
• Consider plasma exchange

Essential Supportive Care

Mandatory prophylaxis for all patients 2, 3:

• Pneumocystis jiroveci: Trimethoprim-sulfamethoxazole 800/160 mg on alternate days or 400/80 mg daily for all patients receiving cyclophosphamide
• Bone protection: Calcium, vitamin D, and bisphosphonates for all patients on long-term glucocorticoids
• Hemorrhagic cystitis prevention: Mesna for patients receiving cyclophosphamide 3

Monitoring Requirements

Regular assessment must include 2, 3:

• Complete blood counts (monitor for leukopenia, especially with cyclophosphamide)
• Renal function and urinalysis
• ANCA levels (persistence or increase may predict relapse and should inform treatment decisions) 2
• Disease activity using BVAS at each visit 2

Treatment Duration

Minimum maintenance therapy duration 1, 3:

• At least 18-24 months for initial remission
• Extended therapy to 4 years after diagnosis reduces relapse risk
• Vasculitis remains a chronic relapsing disorder requiring long-term follow-up 8

Important consideration: Patients with AAV should be treated at centers with experience in AAV management to optimize outcomes 2