How is Primary Angiitis of the Central Nervous System treated? What is the prognosis?
There are no controlled trials. Patients are typically treated with high-dose prednisone and CTX (daily oral or monthly IV) for 3 to 6 months during which time prednisone is tapered to the lowest effective dose. If remission (no new symptoms or MRI lesions) is achieved, patients are switched to maintenance therapy (azathioprine, mycophenolate) for at least another year or longer. Rituximab may be used in patients who are intolerant to CTX. The prognosis is:
- • Mortality 10% to 17%.
- • Moderate to severe disability 20%.
