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Genetic and Rare Diseases Information Center (GARD)

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Primary angiitis of the central nervous system

Other Names for this Disease
  • Granulomatous angiitis of the central nervous system
  • Primary central nervous system vasculitis
  • Primary CNS vasculitis
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Your Question

What medicines have been successfully used other than Cytoxan and Prednisone for primary angiitis of the central nervous system? I've been diagnosed since 2009 and these medicines have compromised my lung.

Our Answer

We have identified the following information that we hope you find helpful. If you still have questions, please contact us.

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

What is primary angiitis of the central nervous system?

Primary angiitis of the central nervous system is a rare form of vasculitis (inflammation of blood vessels) affecting the blood vessels that nourish the brain, spinal cord and peripheral nerves. This condition can lead to narrowing and blockage of the blood vessels of the central nervous system which can eventually cause aneurysms, ischemia and/or hemmorrhage. The cause of this condition is unknown. Signs and symptoms of this condition may begin suddenly or develop over time. Some of the symptoms may incude headaches that do not go away, fever, rapid weight loss, confusion or forgetfulness, and general malaise. Treatment for this condition involves a course of immunosuppresive steroids.[1][3]
Last updated: 10/31/2012