血管炎性末梢神経障害のevidence-based treatment

Evidence-based Treatment for Vasculitic Neuropathies

血管炎性ニューロパチーの治療法に関する知見は限定的であり，全身性血管炎に倣い早期より複合的治療（副腎皮質ステロイド剤＋免疫抑制薬）を行うべきか明らかではない。少なくとも副腎皮質ステロイド剤による単剤治療では，半数近くの症例において効果が不十分とされている。早期に免疫抑制薬の併用が躊躇される際にも，比較的導入しやすい免疫グロブリン静注療法の併用などの治療オプションを早期から活用する工夫が期待される。

Abstract

Vasculitic neuropathies are caused by ischemic damage due to vessel wall inflammation. This damage may cause axonal degeneration leading to permanent neurological disabilities. Therefore, early initiation of effective treatment is crucial. For primary systemic vasculitis, a combined treatment of corticosteroid and immunosuppressive agents is recommended in the evidence-based guidelines as initial standard therapy for induction of remission. However, limited data are available regarding therapies for vasculitic neuropathies and it remains unclear whether combined treatment should be employed as an initial therapy for all patients with vasculitic neuropathies. In approximately half the patients with vasculitic neuropathies, monotherapy with corticosteroids is insufficient in preventing long-lasting neurological disabilities. Addition of intravenous immunoglobulin at an early stage of the disease may be a promising treatment option obviating the need for potent but potentially harmful immunosuppressive agents in some mild or localized cases.