慢性炎症性脱髄性多発ニューロパチーの自己抗体

Autoantibodies in Chronic Inflammatory Demyelinating Polyneuropathy

慢性炎症性脱髄性多発ニューロパチーで自己抗体の報告が相次いでいる。多くはランヴィエ絞輪部周囲の細胞接着分子を標的にするIgG4抗体で，ニューロファシン155とコンタクチン-1に対する抗体の病的意義に関する知見が集約されてきた。これらの抗体が陽性の例は，病理所見で典型的な脱髄像を欠き，免疫グロブリン大量静注療法抵抗性であるなど，一般に本症の特徴とされる所見を示さない。臨床医は自己抗体の意義を理解し，抗体測定を活用することが求められる。

Abstract

Recent reserch has successfully identified several autoantibodies in chronic inflammatory demyelinating polyneuropathy (CIDP). Most of those identified were IgG4 antibodies targeting cell adhesion molecules around the node of Ranvier of myelinated fibers. In particular, much has been learned concerning the pathogenic roles of antibodies against neurofascin 155. Growing evidence has revealed that patients with CIDP with these antibodies are characterized by IVIg-resistance and lack typical demyelinating findings on biopsied nerve fibers, both of which could make diagnosis and treatment difficult without autoantibody measurement. Clinicians should recognize that it is now essential to measure autoantibodies in CIDP and utilize antibody testing.