Primary angiitis of the central nervous system (PACNS): treatment

Written and reviewed by Dr Ibrahim Imam, Consultant Neurologist. Last reviewed .

Primary angiitis of the central nervous system (PACNS): treatment. Methylprednisolone 1g IV daily for 3 days. Fully referenced neurology checklist.

Acute treatment options

  • Methylprednisolone 1g IV daily for 3 days
  • Cyclophosphamide
  • Plasma exchange (PE)

Maintenance treatment options

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Indications for escalating immunosuppression treatment

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References

  1. Rehman HU. Primary angiitis of the central nervous system. J R Soc Med 2000; 93:586-588.
  2. Joseph FG, Scolding NJ. Cerebral vasculitis: a practical approach. Pract Neurol 2002; 2:80-93.
  3. Beuker C, Strunk D, Rawal R, et al. Primary angiitis of the CNS: a systematic review and meta-analysis. Neurol Neuroimmunol Neuroinflamm 2021; 8:e1093.
  4. Paramasivan NK, Sundaram S, Sharma DP, Sreedharan SE, Sylaja PN. Rituximab for refractory primary angiitis of the central nervous system: Experience in two patients. Mult Scler Relat Disord 2021; 51:102907.
  5. Nehme A, Lanthier S, Boulanger M, et al. Diagnosis and management of adult primary angiitis of the central nervous system: an international survey on current practices. J Neurol 2023; 270:1989-1998.

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