Nonsystemic Vasculitic Neuropathy—A Brazilian Case Series

IF 3 3区医学 Q1 CLINICAL NEUROLOGY

Journal of the Peripheral Nervous System Pub Date : 2025-12-09 DOI:10.1111/jns.70086

Victor Evangelista Rodrigues Pereira, Roberto Pereira Santos, Marcos Raimundo Gomes de Freitas, Manuella Lima Gomes Ochtrop, Ana Caroline Siquara-De-Souza, Salim L. Balassiano, Marcia Rodrigues Jardim

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引用次数: 0

Abstract

Background and Aims

Vasculitides are a heterogeneous group of immune-mediated inflammatory disorders that compromise the vascuar wall, leading to luminal narrowing and tissue ischemia. When inflammation selectively affects the vasa nervorum without systemic involvement, it results in nonsystemic vasculitic neuropathy (NSVN), an underrecognized condition. NSVN presents diagnostic challenges due to its variable clinical manifestations and reliance on nerve biopsy for definitive diagnosis. This study aimed to characterize the clinical, neurophysiological, and histopathological features of NSVN in a Brazilian cohort.

Methods

We conducted a cross-sectional, ambispective cohort study combining retrospective chart review and prospective patient assessments. Inclusion required histopathological confirmation of isolated peripheral nerve vasculitis; cases with systemic or secondary vasculitis were excluded. Data collection included clinical evaluation, neurophysiology, and nerve biopsy.

Results

A total of 14 patients were included (9 female, 64%; mean age: 61). Most (n = 8, 57%) had subacute onset of painful sensory or sensorimotor deficits. Multiple mononeuropathies predominated (n = 11, 78%), but a subset exhibited chronic progression (n = 5, 35%) and axonal polyneuropathy (n = 3, 21%). Electrophysiological studies revealed a consistent axonal pattern. Biopsies confirmed possible vasculitis in six (43%), and probable vasculitis in six (42%), with only two (14%) fulfilling criteria for definite vasculitis. Serologies were nonspecific. Treatment involved corticosteroid pulse therapy, with immunosuppression in refractory cases.

Interpretation

These findings highlight that NSVN often presents with painful sensorimotor symptoms and may clinically mimic progressive axonal polyneuropathies. Given its potential for significant morbidity if left untreated, early recognition and consideration of nerve biopsy remain critical. The diagnostic complexity and variability in presentation suggest that NSVN may be underrecognized. We hope this cohort contributes to a broader understanding of its clinical spectrum and informs future diagnostic strategies.

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非全身性血管性神经病-巴西病例系列。

背景和目的：血管炎是一种异质免疫介导的炎症性疾病，损害血管壁，导致管腔狭窄和组织缺血。当炎症选择性地影响血管神经而不累及全身时，它会导致非全身性血管性神经病（NSVN），这是一种未被认识的疾病。NSVN由于其多变的临床表现和对神经活检的明确诊断的依赖，提出了诊断挑战。本研究旨在描述巴西队列中NSVN的临床、神经生理和组织病理学特征。方法：我们进行了一项横断面、双视角队列研究，结合回顾性图表回顾和前瞻性患者评估。孤立性周围神经血管炎需要组织病理学证实；排除全身性或继发性血管炎病例。数据收集包括临床评估、神经生理学和神经活检。结果：共纳入14例患者，其中女性9例，占64%，平均年龄61岁。大多数（n = 8.57%）有亚急性发作的疼痛感觉或感觉运动缺陷。多发性单神经病变占主导地位（n = 11,78%），但一小部分表现为慢性进展（n = 5,35%）和轴突多神经病变（n = 3,21%）。电生理研究显示了一致的轴突模式。活检证实6例（43%）可能有血管炎，6例（42%）可能有血管炎，只有2例（14%）符合明确的血管炎标准。血清学无特异性。治疗包括皮质类固醇脉冲治疗，对难治性病例进行免疫抑制。解释：这些发现强调NSVN通常表现为疼痛的感觉运动症状，并且在临床上可能类似于进行性轴突多发性神经病。如果不及时治疗，其潜在的显著发病率，早期识别和考虑神经活检仍然是至关重要的。诊断的复杂性和表现的可变性表明NSVN可能未被充分认识。我们希望这个队列有助于更广泛地了解其临床谱，并告知未来的诊断策略。

本文章由计算机程序翻译，如有差异，请以英文原文为准。

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来源期刊

Journal of the Peripheral Nervous System 医学-临床神经学

CiteScore

6.10

自引率

7.90%

发文量

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.