Clinical, histologic, and immunologic signatures of Small Fiber Neuropathy in Systemic Lupus Erythematosus

IF 3.9 3区医学 Q1 CLINICAL NEUROLOGY

Journal of the Peripheral Nervous System Pub Date : 2024-07-09 DOI:10.1111/jns.12644

Eleonora Galosi, Carmelo Pirone, Fulvia Ceccarelli, Nicoletta Esposito, Pietro Falco, Martina Leopizzi, Valeria Di Maio, Lorenzo Tramontana, Gianfranco De Stefano, Giuseppe Di Pietro, Giulia Di Stefano, Cristina Garufi, Caterina Leone, Francesco Natalucci, Valeria Orefice, Cristiano Alessandri, Francesca Romana Spinelli, Andrea Truini, Fabrizio Conti

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引用次数: 0

Abstract

Background and Objectives

Systemic Lupus Erythematosus (SLE) often causes damage to small nerve fibers, leading to distressing painful and autonomic symptoms. Despite this, Small Fiber Neuropathy (SFN) remains an underrecognized complication for SLE patients. In this cross-sectional study, we aimed to assess SFN in patients with SLE and to explore its correlations with immunologic disease features and clinical manifestations.

Methods

We recruited 50 SLE patients (1 male to 12.5 females, aged 20–80 years) reporting painful disturbances. We conducted a comprehensive clinical and neurophysiological evaluation, using Nerve Conduction Studies and Quantitative Sensory Testing. Additionally, we carried out an extensive laboratory assessment of disease-related serological parameters. We also performed a thorough skin biopsy analysis, investigating somatic and autonomic innervation while detecting complement and inflammatory cell infiltrates within the skin.

Results

Out of 50 patients, 19 were diagnosed with SFN, primarily characterized by a non-length-dependent distribution; 7 had a mixed neuropathy, with both large and small fiber involvement. Patients with SFN were younger than patients with a mixed neuropathy (p = .0143); furthermore, they were more likely to have a history of hypocomplementemia (p = .0058) and to be treated with cyclosporine A (p = .0053) compared to patients without neuropathy. However, there were no significant differences in painful and autonomic symptoms between patients with and without SFN.

Discussion

This study highlights the relevant frequency of SFN with a non-length-dependent distribution among SLE patients experiencing painful symptoms. Indeed, SFN emerges as an early manifestation of SLE-related neuropathy and is closely associated with hypocomplementemia, suggesting a potential pathogenic role of the complement system. Moreover, SFN may be influenced by disease-modifying therapies. However, the precise role of SFN in shaping painful and autonomic symptoms in patients with SLE remains to be fully elucidated.

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系统性红斑狼疮小纤维神经病的临床、组织学和免疫学特征。

背景和目的：系统性红斑狼疮（SLE）常常会对小神经纤维造成损伤，导致令人痛苦的疼痛和自主神经症状。尽管如此，小神经纤维病变（SFN）仍然是系统性红斑狼疮患者未得到充分认识的并发症。在这项横断面研究中，我们旨在评估系统性红斑狼疮患者的小纤维神经病变，并探讨其与免疫学疾病特征和临床表现的相关性：方法：我们招募了50名报告有疼痛障碍的系统性红斑狼疮患者（男性1人，女性12.5人，年龄20-80岁）。我们使用神经传导研究和定量感觉测试对患者进行了全面的临床和神经生理学评估。此外，我们还对与疾病相关的血清学参数进行了广泛的实验室评估。我们还进行了全面的皮肤活检分析，研究躯体和自主神经支配，同时检测皮肤内的补体和炎性细胞浸润：在 50 名患者中，19 人被确诊为 SFN，其主要特征是非长度依赖性分布；7 人患有混合型神经病，大纤维和小纤维均受累。与混合型神经病变患者相比，SFN 患者更年轻（p = .0143）；此外，与无神经病变患者相比，SFN 患者更可能有低补体血症病史（p = .0058），也更可能接受环孢素 A 治疗（p = .0053）。但是，有SFN和没有SFN的患者在疼痛和自主神经症状方面没有明显差异：讨论：本研究强调了在出现疼痛症状的系统性红斑狼疮患者中，SFN的发生率与长度无关。事实上，SFN 是系统性红斑狼疮相关神经病变的早期表现，与低补体血症密切相关，这表明补体系统具有潜在的致病作用。此外，SFN 还可能受到疾病改变疗法的影响。然而，SFN 在形成系统性红斑狼疮患者疼痛和自主神经症状方面的确切作用仍有待全面阐明。

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.