Cerebral Vasculitis: Diagnosis and Current Treatment Recommendations

The vasculitides are a heterogeneous group of disorders that involve inflammation, necrosis and, ultimately, occlusion of blood vessels. They can have variable CNS involvement, with some, such as isolated angiitis of the CNS (IACNS) and Takayasu’s arteritis, predominantly or exclusively involving the CNS, while others only occasionally cause neurological complications. The latter syndromes include Wegener’s granulomatosis, systemic lupus erythematosus and disorders that mimic vasculitis such as the antiphospholipid antibody syndrome. The treatment of vasculitides is generally with variable doses of corticosteroids and immunosuppressants. Prednisone (oral) and methylprednisolone (usually intravenous) are the most commonly used corticosteroids. Cyclophosphamide is one of the most potent drugs available to treat severe, life-threatening vasculitis. It is given as an oral or intravenous single daily dose regimen or as intravenous pulse therapy. The folate antagonist methotrexate is a useful steroid-sparing agent, and is often added to a corticosteroid regimen. Azathioprine, chlorambucil and cyclosporin have also been used for the treatment of vasculitis, but experience with these agents is limited. Newer therapies with possible, but still unproven, efficacy are intravenous immunoglobulin and monoclonal antibodies. Although general treatment guidelines can be suggested, treatment should be individualised for each vasculitic syndrome.