A small category of Guillain-Barré syndrome (GBS) occurs in the presence of systemic lupus erythematosus (SLE). However, specific treatments for this condition have not been established. Cyclophosphamide (CYC) has been reported to benefit patients with SLE-related GBS in some isolated case reports. Consequently, our objective was to investigate the effectiveness of CYC in SLE-related GBS by means of a systematic literature review. Three online databases, PubMed, Embase and Web of Science, were searched for English articles describing the effectiveness of CYC treatment for SLE-related GBS. We extracted data on patient characteristics, disease course, and CYC efficacy and tolerance. Of 995 studies identified, 26 were included in this systematic review. The data for 28 patients (9 men and 19 women) with SLE-related GBS were reviewed, and the patient age at diagnosis varied from 9 to 72 years (mean: 31.5 years [median: 30.5 years]). Sixteen patients (57.1%) had SLE-related GBS before SLE diagnosis. With regard to CYC response, 24 patients (85.7%) showed resolution (46.4%) or improvement (39.3%) of neurological symptoms. Relapse occurred in one patient (3.6%). Four patients (14.3%) showed no improvement in neurological symptoms following CYC administration. With regard to CYC safety, infections developed in two patients (7.1%), and one death (3.6%) due to posterior reversible encephalopathy syndrome was reported. Lymphopenia developed in one patient (3.6%). Our preliminary data suggest that CYC appears to be an effective treatment for SLE-related GBS. However, it is important to differentiate patients with pure GBS concurrent with SLE, because CYC is ineffective for pure GBS.

Graphical Abstract

中文翻译：

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环磷酰胺治疗系统性红斑狼疮相关格林-巴利综合征：病例报告的系统回顾

一小部分格林-巴利综合征 (GBS) 会在系统性红斑狼疮 (SLE) 的情况下发生。然而，针对这种情况的具体治疗方法尚未确定。据报道，在一些孤立的病例报告中，环磷酰胺 (CYC) 对 SLE 相关 GBS 患者有益。因此，我们的目标是通过系统文献综述来研究 CYC 在 SLE 相关 GBS 中的有效性。检索了三个在线数据库（PubMed、Embase 和 Web of Science），以查找描述 CYC 治疗 SLE 相关 GBS 有效性的英文文章。我们提取了有关患者特征、病程以及 CYC 疗效和耐受性的数据。在确定的 995 项研究中，有 26 项纳入本次系统评价。对 28 名 SLE 相关 GBS 患者（9 名男性和 19 名女性）的数据进行了审查，患者诊断时年龄从 9 岁到 72 岁不等（平均：31.5 岁[中位数：30.5 岁]）。16 名患者 (57.1%) 在 SLE 诊断前患有 SLE 相关 GBS。关于 CYC 反应，24 名患者（85.7%）的神经系统症状得到缓解（46.4%）或改善（39.3%）。1 名患者出现复发（3.6%）。4 名患者（14.3%）在 CYC 给药后神经症状没有改善。关于 CYC 安全性，据报道有 2 名患者 (7.1%) 发生感染，1 名患者因可逆性后部脑病综合征死亡 (3.6%)。一名患者（3.6%）出现淋巴细胞减少症。我们的初步数据表明 CYC 似乎是治疗 SLE 相关 GBS 的有效方法。然而，区分纯 GBS 并发 SLE 的患者很重要，因为 CYC 对纯 GBS 无效。

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