Guillain-Barré症候群と慢性炎症性脱髄性多発ニューロパチーの治療指針

Current therapy of Guillain-Barré syndrome and chronic inflammatory demyelinating polyneuropathy

Guillain-Barré症候群治療の第1選択は，免疫グロブリン大量静注（IVIg）療法である。腎障害のある患者，血栓・塞栓症の危険性の高い患者には，単純血漿交換療法を選択する。置換液としてアルブミン液を用いる。5m以上歩ける軽症例では隔日で2回の単純血漿交換療法が，自分で立てない中等度例や人工呼吸器に装着されている重症例では隔日で4回の単純血漿交換療法が適当である。慢性炎症性脱髄性多発ニューロパチーでは，IVIgを第1選択とすべきである。初回IVIに反応した時点でprednisoloneを開始する。初回IVIg開始日の4週後に2回目のIVIgを追加する。Prednisolone漸減中に再燃した場合，速やかにIVIgを開始し免疫抑制剤に切り換える。

The first choice to treat patients with Guillain-Barré syndrome is intravenous immunoglobulin (IVIg) therapy. Patients with renal dysfunction and a risk of thromboembolism should be treated by plasma exchange. Not fresh frozen plasma but diluted albumin should be used as the replacement fluid. Patients with Guillain-Barré syndrome who can walk more than 5 m without assistance should receive 2 plasma exchanges. Patients who cannot stand alone or are mechanically ventilated should benefit from 4 exchanges. The first choice to treat patients with chronic inflammatory demyelinating polyneuropathy is IVIg. Prednisolone should be started as soon as the muscle weakness begin to lessen. The second course of IVIg should be given after 4 weeks of the first course. When the weakness recur during the tapering prednisolone, the patients should receive another course of IVIg followed by an immunosuppressive agent such as cyclosporin.