2021年EAN/PNS CIDPガイドラインを紐解く

Updated Diagnosis and Treatment for Chronic Inflammatory Demyelinating Polyradiculoneuropathy Based on the EAN/PNS Guideline 2021

2021年に欧州神経学会（EAN）と国際末梢神経学会（PNS）によるCIDPの診断・治療ガイドラインが公表された。2005年初版，2010年改訂版に続く第3版となる。この間に疾患概念の整理，電気診断・末梢神経画像診断の進歩，傍絞輪部蛋白に対する新規抗体の発見があり，治療の面からは免疫グロブリン維持療法が開発され，新ガイドラインではこれらを包括した改訂が行われた。CIDPの中心病型は対称性に近位筋・遠位筋がともに障害される典型的CIDPであり，旧版で非典型的CIDPとされていた遠位型・多巣型なども病型はvariantとされ別個に診断基準が示され，狭義のCIDPには入らないニュアンスが示された。本論では改訂点のポイントとともに現状で残された問題点についてタスクフォース委員間で議論されながら記載に至らなかった臨床病型の定義・疾患概念などの問題点を含めて概説する。

Abstract

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) includes a number of clinical subtypes. The major phenotype is “typical CIDP,” which is characterized by symmetric and “proximal and distal” muscle weakness. Due to historical changes in the concept of CIDP, multifocal motor neuropathy, anti-myelin-associated glycoprotein (anti-MAG) neuropathy, and autoimmune nodopathy were excluded. International guidelines for the diagnosis and treatment of CIDP were first published in 2005 and revised in 2010. In 2021, the guidelines of the European Academy of Neurology (EAN)/Peripheral Nerve Society (PNS) were the second revision, reflecting changes in the disease concept and progress of electrodiagnosis, neuroimaging, and novel treatments. This review introduces the outline of the guidelines in addition to typical CIDP; related chronic demyelinating neuropathies were classified as CIDP variants. The diagnosis of CIDP is based on (1)the phenotype of a typical CIDP or variant, (2)electrophysiological evidence of peripheral nerve demyelination, and (3)exclusion criteria. The first-line treatments for typical CIDP are corticosteroids and immunoglobulin therapy. These guidelines recommend intravenous or subcutaneous immunoglobulin as maintenance therapy, as well as unresolved questions on the evolving concept of CIDP and future treatments.