CIDP治療の歩み—症状改善を目的とした治療から維持療法へ〜SCIgを中心に〜

A Review of the History of CIDP Treatment: From Symptom Improvement to Maintenance Therapy- with a Focus on SCIg

慢性炎症性脱髄性多発根ニューロパチー（CIDP）の治療に関する臨床研究は，2000年代前半までの初発・再発時における症状改善のための治療，2000年代後半以降の病勢進行の抑制を目的とする維持療法，さらに近年では個々の治療法の最適化へとコンセプトが移ってきた。 本総説ではCIDP治療に関わる過去の臨床研究を振り返り，これまでの変遷を概観する。そのうえでそれぞれの治療法の位置付けを明らかにし，課題と今後の治療開発の方向性について述べる。

Abstract

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is an autoimmune peripheral neuropathy with chronic progression over 2 months or more. In this review, we provide an overview of key clinical studies involved in the development of CIDP therapy, as well as a discussion of changes in the concept of treatment. Although a definitive therapy has not yet been established, international and Japanese clinical guidelines recommend three first-line treatment options for symptom improvement, namely corticosteroids, intravenous immunoglobulin (IVIg), and plasmapheresis, based on the results of clinical studies conducted before the early 2000s. Since 2010, several treatments for the prevention of CIDP relapse (maintenance therapy) have been developed and more recently, studies have focused on the optimization of each treatment. On the other hand, CIDP treatment is associated with several limitations, including a lack of biomarkers for prediction of disease progression, differences in response to treatment between CIDP subtypes, and difficulties in the selection of appropriate maintenance therapy. Several studies aimed at resolving these issues are currently being conducted.

(Received 22 May, 2020; Accepted 12 January, 2021; Published 1 July, 2021)