What is the treatment for Central Nervous System (CNS) vasculitis?

Treatment of Central Nervous System (CNS) Vasculitis

The standard treatment for CNS vasculitis consists of induction therapy with high-dose glucocorticoids plus cyclophosphamide, which has an 80% response rate, followed by maintenance immunosuppression therapy. 1

Classification and Approach to Treatment

CNS vasculitis can be categorized into:

1. Primary CNS vasculitis - limited to the brain and spinal cord
2. Secondary CNS vasculitis - part of systemic conditions or other causes

Treatment Algorithm

Induction Therapy (First 3-6 months)

• First-line treatment:

  • High-dose glucocorticoids (typically IV methylprednisolone 1g/day for 3-5 days) 2
  • Followed by oral prednisone (1 mg/kg/day, maximum 60-80 mg/day) 3
  • PLUS cyclophosphamide (either):
    • Oral: 2 mg/kg/day (maximum 200 mg/day) 3
    • IV pulse: 15 mg/kg every 2-4 weeks (preferred due to lower cumulative dose) 3

• For severe/life-threatening disease:

  • More aggressive immunosuppression may be needed 4
  • Consider plasma exchange in rapidly progressive cases

Maintenance Therapy (1-2+ years)

After achieving remission (typically 3-6 months):

• Gradually taper glucocorticoids
• Switch from cyclophosphamide to less toxic agents:
  • Azathioprine (2 mg/kg/day)
  • Methotrexate (15-25 mg/week)
  • Mycophenolate mofetil (2-3 g/day)

Alternative Therapies

• Rituximab: May be considered as an alternative to cyclophosphamide, particularly in:

  • Patients intolerant to cyclophosphamide
  • Refractory disease
  • ANCA-associated vasculitis with CNS involvement 5, 6

• For secondary CNS vasculitis:

  • Treatment should address the underlying cause 1
  • For VZV-associated CNS vasculitis: IV aciclovir plus corticosteroids 3
  • For HCV-associated cryoglobulinemic vasculitis: Consider rituximab 3

Monitoring and Supportive Care

• Regular clinical assessment for neurological improvement

• Laboratory monitoring:

  • Complete blood count (weekly initially)
  • Renal and liver function tests
  • Urinalysis

• Prophylaxis:

  • Pneumocystis jirovecii pneumonia prophylaxis with trimethoprim/sulfamethoxazole for patients on cyclophosphamide 3
  • Consider MESNA with cyclophosphamide to prevent hemorrhagic cystitis 3
  • Bone protection for patients on long-term steroids

Prognostic Factors

Poor prognostic factors include:

• Increasing age at diagnosis
• Large vessel involvement
• Cerebral infarcts at presentation
• Diagnosis by angiography alone 4

Better outcomes are associated with:

• Gadolinium-enhanced cerebral lesions
• Prompt diagnosis and early treatment 4

Special Considerations

• Small vessel vasculitis: Often presents with cognitive deficits, altered consciousness, and seizures
• Medium vessel vasculitis: Typically presents with focal neurological deficits and stroke-like episodes
• Refractory disease: Consider rituximab or other biological agents after failure of standard therapy 6

Brain biopsy remains the gold standard for definitive diagnosis, and treatment should be initiated promptly when clinical suspicion is high, even before confirmatory pathologic testing in unstable patients 1.