10年后自身免疫性淋巴结副病:临床特征、病理生理和治疗

IF 3.9 3区 医学 Q1 CLINICAL NEUROLOGY Journal of the Peripheral Nervous System Pub Date : 2023-06-05 DOI:10.1111/jns.12569
Antonino Uncini
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引用次数: 0

摘要

背景和目的基于病理生理学,自身免疫性神经病分为脱髓鞘性和轴突性。2013年,为了更好地对含有抗神经节苷脂抗体的神经病进行分类,引入了“淋巴结-副神经节病”这一术语,后来扩大到包括含有针对淋巴结和副神经节轴胶质复合物抗体的神经病,这一术语描述了淋巴结区域在发病机制中起关键作用的疾病。这些神经病变,虽然表现出电生理脱髓鞘特征,但没有表现出节段性脱髓鞘的病理证据,或者,虽然被归类为轴突性,但可以表现出可逆的神经传导障碍和快速恢复,这与轴突性神经病变的普遍概念相反。方法根据周围神经纤维、靶抗原、所涉及的抗体类别和亚类别对自身免疫性结节-副病理进行分类,采用分裂方法报道自身免疫性结节-副病理的最新进展。并对其临床特点、电生理表现、免疫病理及超微结构检查结果、病理生理及治疗进行了叙述。结果与解释结节-副神经病变分类整合了自身免疫性神经病变的临床分类,扩展了传统的脱髓鞘和轴突分类。它有助于更好地系统化指出自身免疫过程的域和靶抗原,它解决了相互矛盾的病理和电生理发现,调和了轴突神经病变可能迅速可逆的矛盾,避免了分类混乱和可能的误诊。最后,通过鉴定特异性抗体及其流行类和亚类,这种分类澄清了病理生理机制,并提出了更有针对性的治疗方法。
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Autoimmune nodo-paranodopathies 10 years later: Clinical features, pathophysiology and treatment

Background and Aims

Autoimmune neuropathies are classified, on the basis of pathophysiology, as demyelinating or axonal. The term nodo-paranodopathy, introduced in 2013 to better categorize the neuropathies with antiganglioside antibodies and later expanded to include neuropathies with antibodies to nodal and paranodal axoglial complexes, characterizes disorders in which the nodal region is critical in the pathogenesis. These neuropathies, although presenting electrophysiologic demyelinating features do not show pathologic evidence of segmental demyelination, or, although being classified as axonal, can show reversible nerve conduction failure and rapid recovery contrary with the communal concept of an axonal neuropathy.

Methods

In this personal view is reported, with a splitting approach, an update on autoimmune nodo-paranodopathies, classified according to the domains of peripheral nerve fiber, the target antigens and the antibody class and subclass involved. The clinical features, the electrophysiologic findings, the results of the immunopathological and ultrastructural studies, the pathophysiology and treatment are also described.

Results and Interpretation

The nodo-paranodopathy category integrates the clinical classification of autoimmune neuropathies and expands the traditional dichotomous demyelinating and axonal classification. It helps to a better systematization pointing to the domain and target antigens of the autoimmune process, it resolves conflicting pathologic and electrophysiologic findings, reconciles the contradiction that axonal neuropathies may be rapidly reversible, avoids taxonomical confusion and possible misdiagnoses. Finally this categorization, through the identification of the specific antibody and its prevalent class and subclass, clarifies the pathophysiological mechanisms and addresses to a more targeted therapeutic approach.

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来源期刊
CiteScore
6.10
自引率
7.90%
发文量
45
审稿时长
>12 weeks
期刊介绍: The Journal of the Peripheral Nervous System is the official journal of the Peripheral Nerve Society. Founded in 1996, it is the scientific journal of choice for clinicians, clinical scientists and basic neuroscientists interested in all aspects of biology and clinical research of peripheral nervous system disorders. The Journal of the Peripheral Nervous System is a peer-reviewed journal that publishes high quality articles on cell and molecular biology, genomics, neuropathic pain, clinical research, trials, and unique case reports on inherited and acquired peripheral neuropathies. Original articles are organized according to the topic in one of four specific areas: Mechanisms of Disease, Genetics, Clinical Research, and Clinical Trials. The journal also publishes regular review papers on hot topics and Special Issues on basic, clinical, or assembled research in the field of peripheral nervous system disorders. Authors interested in contributing a review-type article or a Special Issue should contact the Editorial Office to discuss the scope of the proposed article with the Editor-in-Chief.
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