3種類のmainstay治療に反応しないCIDP—適切な寛解導入療法と治療効果判定

CIDP Refractory to Three Types of Mainstay Treatments: Appropriate Induction Therapy and Evaluation of Therapeutic Effect

慢性炎症性脱髄性多発根ニューロパチー（CIDP）はheterogeneousな病態を持つ疾患であり，mainstay治療への反応性もCIDPの病型で異なる。CIDPの病型，治療法の特性，患者背景を基に治療法を選択する。Mainstay治療抵抗性CIDPでは，寛解導入療法が適切に行われ，客観的指標で治療効果が適切に評価されたかどうかを確認する。治療抵抗性multifocal CIDP，distal CIDPでは慎重な鑑別診断が必要である。

Abstract

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is a disease with a heterogeneous pathology. The responsiveness to mainstay treatment differs depending on the type of CIDP. The treatment strategy is determined based on the type of CIDP, characteristics of the therapeutic agents and treatment methods, and patient background. For CIDPs that do not respond to the mainstay treatment, it is necessary to review whether the induction treatment was properly performed and whether the therapeutic effect was properly evaluated using objective indicators. In particular, treatment-resistant multifocal CIDP and distal CIDP require careful differential diagnoses. Intervention trials using rituximab and anti-neonatal Fcγ receptor monoclonal antibodies are underway, and it is expected that clear treatment guidelines will soon be developed for refractory CIDP.