What is the treatment for vasculitis?

Treatment of Vasculitis

For severe systemic vasculitis, initiate combination therapy immediately with cyclophosphamide (2 mg/kg/day oral or IV pulses) plus high-dose glucocorticoids (prednisolone 1 mg/kg/day, maximum 60 mg/day), or alternatively rituximab (375 mg/m² weekly for 4 weeks) plus glucocorticoids, with rituximab preferred for relapsing disease or fertility concerns. 1, 2, 3

Disease Categorization and Severity Assessment

• Patients must be categorized by vessel size and disease severity before treatment initiation to determine appropriate therapeutic intensity 1, 2, 3
• For ANCA-associated vasculitis (AAV), classify as: localized, early systemic, generalized, severe, or refractory disease 3
• Severe disease indicators include: rapidly progressive renal failure, diffuse alveolar hemorrhage, or other organ-threatening manifestations 3
• Management should occur at centers with vasculitis expertise 1, 2

Induction Therapy by Disease Type

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

For generalized/severe disease:

• Cyclophosphamide 2 mg/kg/day oral (maximum 200 mg/day) OR intravenous pulses PLUS prednisolone 1 mg/kg/day (maximum 60 mg/day) 1, 2
• Rituximab 375 mg/m² weekly for 4 weeks is equally effective as cyclophosphamide, achieving 64% complete remission at 6 months versus 53% with cyclophosphamide 2, 4
• Rituximab is preferred over cyclophosphamide for: younger patients with fertility concerns (67% vs 42% remission rate in relapsing disease) and patients with relapsing disease 3, 4
• For markedly reduced or rapidly declining renal function, consider combination of rituximab AND cyclophosphamide 3

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

• Methotrexate (oral or parenteral) plus glucocorticoids as a less toxic alternative 1
• Methotrexate should NOT be used if GFR <60 mL/min per 1.73 m² 3

Critical adjunctive measures:

• Provide Pneumocystis jiroveci prophylaxis (co-trimoxazole) during cyclophosphamide therapy 2
• Use Mesna as uroprotective agent with cyclophosphamide, though this does not eliminate bladder cancer risk entirely 1, 2
• Plasma exchange is recommended for patients with dialysis requirement, rapidly progressive renal failure, or diffuse alveolar hemorrhage with hypoxemia 1, 3

Large Vessel Vasculitis (Giant Cell Arteritis, Takayasu Arteritis)

• Initiate high-dose glucocorticoids immediately (prednisolone 1 mg/kg/day, typically 40-60 mg/day) 2, 3
• Add methotrexate (10-15 mg/week for giant cell arteritis, 20-25 mg/week for Takayasu arteritis) as steroid-sparing agent to reduce relapse rate and cumulative glucocorticoid dose 2
• Prescribe low-dose aspirin (75-150 mg/day) for all giant cell arteritis patients to prevent cardiovascular and cerebrovascular events 2
• For Takayasu arteritis, all patients should receive non-biological glucocorticoid-sparing agents in combination with glucocorticoids 3

Polyarteritis Nodosa and Churg-Strauss Syndrome

• Cyclophosphamide plus glucocorticoids for remission induction 1
• For hepatitis B-associated PAN: combination of antiviral therapy, plasma exchange, and glucocorticoids 1

Cryoglobulinemic Vasculitis

• For hepatitis C-associated: antiviral therapy 1
• For essential (hepatitis C negative): immunosuppressive therapy, managed in conjunction with hepatologist 1

Maintenance Therapy

Once remission is achieved (typically after 3-6 months):

• Transition to low-dose glucocorticoids (5-7.5 mg/day) PLUS one of the following: 1, 3

  • Azathioprine (first-line, strongest evidence) 1
  • Rituximab (preferred for relapsing disease) 3
  • Leflunomide 1
  • Methotrexate (if GFR ≥60 mL/min per 1.73 m²) 1, 3
  • Mycophenolate mofetil 1, 3

• Minimum maintenance duration: 18-24 months for initial remission 3

• Extended therapy to 4 years after diagnosis reduces relapse risk 3

• Glucocorticoid maintenance should continue at 5-7.5 mg/day for 2 years, then reduce by 1 mg every 2 months 3

Refractory Disease Management

For patients failing standard therapy or experiencing relapse:

• Rituximab achieves 91% remission rate in refractory AAV (42/46 patients in open-label trials) 1, 4
• Intravenous immunoglobulin (IVIG) 2 gm/kg for short-term control 1, 2
  • Must measure serum immunoglobulin levels first due to anaphylaxis risk in IgA deficiency 1, 2
• Alternative options: mycophenolate mofetil, 15-deoxyspergualin, anti-thymocyte globulin, infliximab 1
• Refer to expert center for enrollment in clinical trials 1

Monitoring Requirements

• Structured clinical assessment, urinalysis, and basic laboratory tests at EVERY clinical visit 1, 2
• Monitor with ESR, CRP, comprehensive metabolic panel 3
• Investigate persistent unexplained hematuria in ALL patients with prior cyclophosphamide exposure due to bladder cancer risk (can occur months to years after discontinuation) 1, 2
• Use Birmingham Vasculitis Activity Score (BVAS) for disease activity assessment 3
• For Takayasu arteritis, consider periodic MRI or PET imaging for disease activity 2

Critical Pitfalls to Avoid

• Do NOT delay treatment while waiting for biopsy results in rapidly deteriorating patients 3
• Tobacco smokers on cyclophosphamide develop bladder cancer at lower doses and earlier than non-smokers 1
• Do NOT use alternate-day glucocorticoid therapy (increases relapse risk) 3
• Inadequate initial immunosuppression in severe disease leads to permanent organ damage or death 2, 3
• Untreated systemic vasculitis carries 40-46% five-year mortality in patients with poor prognostic factors 2