自身免疫性结节病

Q4 Immunology and Microbiology
Masahiro Iijima
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引用次数: 0

摘要

自身免疫性结节病(AN)的特点是存在针对髓鞘神经盐传导所必需的分子的自身抗体。自身免疫性结节病的临床表现与慢性炎症性脱髓鞘多发性神经病(CIDP)相似,包括进行性和对称性感觉运动障碍,在神经传导研究中具有电生理脱髓鞘特征。虽然在 CIDP 中尚未发现常见的自身抗体,但 AN 的特点是自身抗体主要针对以 Ranvier 节点和副节点为代表的特定分子复合结构。此外,神经筋膜素-155(NF155)、接触素-1(CNTN1)、接触素相关蛋白1(Caspr1)和CNTN1/Caspr1复合物等自身抗体就是此类自身抗体的典型例子。它们会破坏副结节的隔膜样连接,而不会引发细胞免疫反应。共济失调、震颤、脑脊液(CSF)蛋白水平明显增高等症状是自发性神经病的独特表现,通常还伴有神经根和颅神经肥大。值得注意的是,这种疾病对通常对 CIDP 有效的免疫疗法(包括静脉注射免疫球蛋白疗法(IVIg))具有抗药性。目前的证据表明,B 细胞清除疗法(如利妥昔单抗)可能有益于 AN 的治疗。由于有研究表明,现有的 CIDP 治疗方法可能对 IgG4 以外亚类的自身抗体阳性病例有效,因此对传统疗法耐药的 CIDP 需要考虑到 IgG4 自身抗体可能性的新型治疗策略。
本文章由计算机程序翻译,如有差异,请以英文原文为准。
Autoimmune nodopathy

Autoimmune nodopathy (AN) is characterized by the presence of autoantibodies targeting molecules essential for saltatory conduction in myelinated nerves. Clinical manifestations of AN show similarities with chronic inflammatory demyelinating polyradiculoneuropathy (CIDP), including progressive and symmetrical sensorimotor deficits with electrophysiological demyelinating features in nerve conduction studies. Although no common autoantibodies have yet been identified in CIDP, AN is characterized by autoantibodies that primarily target specific molecular complex structures represented by the Ranvier node and paranode. Furthermore, these autoantibodies, such as neurofascin-155 (NF155), contactin-1 (CNTN1), contactin-related protein 1 (Caspr1), and the CNTN1/Caspr1 complex, are illustrative examples of such autoantibodies. They can disrupt the septal-like junction of paranodes without triggering cellular immune responses. AN manifests uniquely with symptoms such as ataxia, tremors, and markedly high cerebrospinal fluid (CSF) protein levels, often accompanied by nerve root and cranial nerve hypertrophy. Notably, this condition is resistant to immunotherapies typically effective against CIDP, including intravenous immunoglobulin therapy (IVIg). Current evidence suggests that B-cell depletion therapies, such as rituximab, could benefit AN treatment. Since it has been suggested that existing treatments for CIDP may be effective in cases of autoantibody positivity of subclasses other than IgG4, CIDP that is resistant to conventional therapy requires novel therapeutic strategies that take into account the possibility of IgG4 autoantibodies.

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来源期刊
Clinical and Experimental Neuroimmunology
Clinical and Experimental Neuroimmunology Immunology and Microbiology-Immunology and Microbiology (miscellaneous)
CiteScore
1.60
自引率
0.00%
发文量
52
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