Wolbert Jolien, Cheng Mandy I, Meyer zu Horste Gerd, Su Maureen A
Department of Neurology with Institute of Translational Neurology, University Hospital Münster, Münster, Germany.
Department of Microbiology Immunology and Medical Genetics and.
JCI Insight. 2020 Feb 13;5(3):132411. doi: 10.1172/jci.insight.132411.
Chronic inflammatory demyelinating polyneuropathy (CIDP) is an autoimmune disease of the peripheral nerves that presents with either chronic progression or relapsing disease. Recent studies in samples from patients with CIDP and mouse models have delineated how defects in central (thymic) and peripheral (extrathymic) immune tolerance mechanisms can cause PNS autoimmunity. Notably, nerve parenchymal cells actively contribute to local autoimmunity and also control disease outcome. Here, we outline how emerging technologies increasingly enable an integrated view of how immune cells and PNS parenchymal cells communicate in CIDP. We also relate the known heterogeneity of clinical presentation with specific underlying mechanisms. For example, a severe subtype of CIDP with tremor is associated with pathogenic IgG4 autoantibodies against nodal and paranodal proteins. An improved understanding of pathogenic mechanisms in CIDP will form the basis for more effective mechanism-based therapies.
慢性炎症性脱髓鞘性多发性神经病（CIDP）是一种周围神经的自身免疫性疾病，表现为慢性进展或复发。最近对CIDP患者样本和小鼠模型的研究已经阐明了中枢（胸腺）和外周（胸腺外）免疫耐受机制的缺陷如何导致周围神经系统自身免疫。值得注意的是，神经实质细胞积极参与局部自身免疫，并控制疾病结局。在这里，我们概述了新兴技术如何越来越能够让人们全面了解免疫细胞和周围神经系统实质细胞在CIDP中如何相互作用。我们还将已知的临床表现异质性与特定的潜在机制联系起来。例如，一种伴有震颤的严重CIDP亚型与针对结旁和节旁蛋白的致病性IgG4自身抗体有关。对CIDP致病机制的更好理解将为更有效的基于机制的治疗奠定基础。
