中枢神経血管炎の治療戦略

Treatment for Central Nervous System Vasculitis

中枢神経血管炎は，脳や脊髄に限局するprimary central nervous system(CNS) vasculitisと，感染症や全身性疾患による二次性のものに分類される。Primary CNS vasculitisの治療方針を立てるためには何よりも正確な診断が重要であり，脳組織所見や脳血管造影所見に加えて，二次性血管炎の除外を目的とした全身検索が必要とされる。Primary CNS vasculitisの疾患概念が提唱された当初は治療抵抗性で予後不良と考えられていたが，免疫抑制薬の併用などにより良好な転帰が得られる症例が少なくないことがわかってきた。本稿ではprimary CNS vasculitisに対する治療を中心に概説する。

Abstract

Central nervous system (CNS) vasculitis is classified into two categories based on whether it is primary or secondary. Primary CNS vasculitis is rare disorder of unknown cause that is restricted to the brain and spinal cord. Currently, there are no randomized clinical trials for treatment of primary CNS vasculitis. Therefore, treatment regimens for primary CNS vasculitis have been derived from therapeutic strategies used in other kinds of vasculitis. Early detection is important because corticosteroid treatment with or without cyclophosphamide can often prevent serious outcomes and may result in a favorable response. However, because some patients are intolerant or respond poorly to combination therapy, development of novel treatment options is eagerly awaited. Several immunosuppressive agents such as mycophenolate mofetil, tumor necrosis factor-α blockers, and rituximab could be options for refractory patients.