Abstract：
OBJECTIVE: 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.
CONCLUSIONS: 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.
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.
CONCLUSIONS: 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.
摘要：
目的：系统性红斑狼疮（SLE）常引起小神经纤维损伤，导致痛苦和自主神经症状。尽管如此,小纤维神经病变（SFN）仍然是SLE患者未被认识到的并发症。在这项横断面研究中，我们旨在评估SLE患者的SFN,并探讨其与免疫疾病特征和临床表现的相关性.
方法：我们招募了50名SLE患者（1名男性至12.5名女性，20-80岁)报告痛苦的干扰。我们进行了全面的临床和神经生理学评估，使用神经传导研究和定量感觉测试。此外,我们对疾病相关血清学参数进行了广泛的实验室评估.我们还做了彻底的皮肤活检分析,研究躯体和自主神经支配，同时检测皮肤内的补体和炎症细胞浸润。
结果：在50名患者中，19人被诊断为SFN，主要特征为非长度依赖性分布；7患有混合性神经病，涉及大纤维和小纤维。SFN患者比混合性神经病患者年轻(p=.0143)；此外，与无神经病变的患者相比,他们更可能有低补体血症病史(p=.0058),并且更可能接受环孢素A治疗(p=.0053).然而,有和没有SFN的患者在疼痛和自主神经症状方面没有显著差异.
结论：本研究强调了在出现疼痛症状的SLE患者中，非长度依赖性分布的SFN的相关频率。的确,SFN是SLE相关神经病变的早期表现，与低补体血症密切相关。提示补体系统的潜在致病作用。此外，SFN可能受到疾病改善疗法的影响。然而,SFN在SLE患者疼痛和自主神经症状形成中的确切作用仍有待完全阐明.
方法：我们招募了50名SLE患者（1名男性至12.5名女性，20-80岁)报告痛苦的干扰。我们进行了全面的临床和神经生理学评估，使用神经传导研究和定量感觉测试。此外,我们对疾病相关血清学参数进行了广泛的实验室评估.我们还做了彻底的皮肤活检分析,研究躯体和自主神经支配，同时检测皮肤内的补体和炎症细胞浸润。
结果：在50名患者中，19人被诊断为SFN，主要特征为非长度依赖性分布；7患有混合性神经病，涉及大纤维和小纤维。SFN患者比混合性神经病患者年轻(p=.0143)；此外，与无神经病变的患者相比,他们更可能有低补体血症病史(p=.0058),并且更可能接受环孢素A治疗(p=.0053).然而,有和没有SFN的患者在疼痛和自主神经症状方面没有显著差异.
结论：本研究强调了在出现疼痛症状的SLE患者中，非长度依赖性分布的SFN的相关频率。的确,SFN是SLE相关神经病变的早期表现，与低补体血症密切相关。提示补体系统的潜在致病作用。此外，SFN可能受到疾病改善疗法的影响。然而,SFN在SLE患者疼痛和自主神经症状形成中的确切作用仍有待完全阐明.
