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Diseases

Genetic and Rare Diseases Information Center (GARD)

Primary angiitis of the central nervous system


Other Names for this Disease
  • PACNS
  • Primary central nervous system vasculitis
  • Primary CNS vasculitis
  • Granulomatous angiitis of the central nervous system
  • Isolated angiitis of the central nervous system
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Treatment

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How might primary angiitis of the central nervous system be treated? 

The current treatment recommendation is to start with oral prednisone at a dose of 1 mg/kg per day and cyclophosphamide at a dose of 2 mg/kg per day. Most centers use prednisone and cyclophosphamide for 4-6 months to induce clinical remission, and then taper prednisone off.[1] Patients generally stay on cyclophosphamide therapy between three and six months, depending on when remission occurs and if there are any potential side effects from cyclophosphamide. Once cyclophosphamide is discontinued, it should be replaced with a less toxic medication for an additional six to twelve months of maintenance therapy. Some doctors switch from cyclophosphamide to azathioprine (2 mg/kg) or mycophenolate mofetil. Methotrexate can also be used, but may be limited by its difficulty to cross the blood brain barrier. There is limited data on how long the maintenance therapy lasts so the decision on the duration of the therapy should be individualized, based upon how the patient responds to therapy. [2]
Last updated: 10/31/2012

References
  1. Noufal M. Primary Angiitis of the CNS. Medscape Reference. April 2012; http://emedicine.medscape.com/article/1484146-treatment. Accessed 10/29/2012.
  2. Hajj-Ali, R., Calabrese L. Primary angiitis of the centeral nervous system. UpToDate. April 18, 2012; http://www.uptodate.com/contents/primary-angiitis-of-the-central-nervous-system#H30. Accessed 10/29/2012.


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Other Names for this Disease
  • PACNS
  • Primary central nervous system vasculitis
  • Primary CNS vasculitis
  • Granulomatous angiitis of the central nervous system
  • Isolated angiitis of the central nervous system
See Disclaimer regarding information on this site. Some links on this page may take you to organizations outside of the National Institutes of Health.