CIDPではなくなった疾患—抗MAGニューロパチーと自己免疫性ノドパチー

Diseases Separated from Chronic Inflammatory Demyelinating Polyradiculoneuropathy: Anti-Myelin-Associated Glycoprotein Neuropathy and Autoimmune Nodopathy

抗MAGニューロパチーと絞輪部・傍絞輪部蛋白抗体が陽性の自己免疫性ノドパチーは近年CIDPから分離され，独立した疾患として位置づけられた。自己抗体陽性例の臨床的均一性やCIDPと治療反応性が異なる点，自己抗体の病原性の証拠が分離の理由である。CIDPの括りから外れる一群を見出す意義は，個別の病態を明らかにし，適切な免疫治療を施すことにある。将来，CIDPが有する多様な病態が明らかにされ，病態の異なるCIDP亜型がなくなることを期待したい。

Abstract

Anti-myelin-associated glycoprotein (Anti-MAG) neuropathy and autoimmune nodopathies with antibodies targeting nodal or paranodal proteins have recently been reclassified as distinct conditions, separate from chronic inflammatory demyelinating polyradiculoneuropathy (CIDP). This distinction is based on the clinical homogeneity observed in antibody-positive cases, their unique response to treatment compared to CIDP, and evidence indicating the pathogenic role of these autoantibodies. The significance of identifying conditions outside the CIDP category lies in the elucidation of their distinct pathological mechanisms and providing appropriate immunotherapy accordingly. We hope that the various pathologies currently grouped under CIDP will be further clarified in the future, leading to the elimination of CIDP variants with different pathophysiologies.