Efficacy of tumor necrosis factor alpha blockade in primary central nervous system vasculitis resistant to immunosuppressive treatment.

摘要

Primary central nervous system vasculitis (PCNSV) is an uncommon form of vasculitis that is limited to the brain and spinal cord. PCNSV may be associated with serious morbidity and mortality unless it is treated with high-dose corticosteroids and immunosuppressive medications (1-3).Because of the lack of controlled clinical trials and uniform diagnostic criteria, uncertainties exist regarding the response to treatment and the long-term outcome of PGNSV. Some patients may have a more benign course and may need less intense therapy (4,5). Others require more aggressive therapy. Still others experience recurrent symptoms or have severe adverse effects, therefore new treatment options are needed (6-9).We report 2 patients with PCNSV who did not respond to treatment with corticosteroids and immunosuppressive medication. They were treated with tumor necrosis factor (TNF) blockers in an attempt to control the disease. This study was approved by the Mayo Clinic Institutional Review Board.