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Distinguish CIDP with autoantibody from that without autoantibody: pathogenesis, histopathology, and clinical features

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Abstract

Chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) is considered to be an immune-mediated heterogeneous disease involving cellular and humoral immunity. In recent years, autoantibodies against nodal/paranodal protein neurofascin155 (NF155), neurofascin186 (NF186), contactin-1 (CNTN1), and contactin-associated protein 1 (CASPR1) have been identified in a small subset of patients with CIDP, which disrupt axo-glial interactions at nodes/paranodes. Although CIDP electrodiagnosis was made in patients with anti-nodal/paranodal component autoantibodies, macrophage-induced demyelination, the characteristic of typical CIDP, was not observed. Apart from specific histopathology, the pathogenic mechanisms and clinical manifestations of CIDP with autoantibody are also distinct. We herein compared pathogenesis, histopathology, clinical manifestations, and therapeutic response in CIDP with autoantibody vs. CIDP without autoantibody. CIDP with autoantibodies should be considered as an independent disease entity, not a subtype of CIDP due to many differences. They possibly should be classified as CIDP-like chronic nodo-paranodopathy, which can better characterize these disorders, help diagnose and make the most effective therapeutic decisions.

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Acknowledgements

The authors would like to thank Yi Shan for his dedication to the figure for this paper.

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Authors and Affiliations

  1. Department of Neurology, The Second Xiangya Hospital, Central South University, Renmin Road 139#, Changsha, 410011, Hunan, China

    Lisha Tang, Qianyi Huang, Zhen Qin & Xiangqi Tang

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  1. Lisha Tang
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  2. Qianyi Huang
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  3. Zhen Qin
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  4. Xiangqi Tang
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L.T. developed the concept and design of the review, and drafted the manuscript for intellectual content. Q.H. and Z.Q. revised the manuscript for intellectual content. X.T. supervised the work and contributed to the review revision.

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Correspondence to Xiangqi Tang.

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Tang, L., Huang, Q., Qin, Z. et al. Distinguish CIDP with autoantibody from that without autoantibody: pathogenesis, histopathology, and clinical features. J Neurol 268, 2757–2768 (2021). https://doi.org/10.1007/s00415-020-09823-2

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  • DOI: https://doi.org/10.1007/s00415-020-09823-2

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