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Cyclophosphamide in the Treatment of Systemic Lupus Erythematosus-related Guillain-Barré Syndrome: A Systematic Review of Case Reports

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Abstract

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.

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Funding

This work was supported by the Government and School Cooperation Program of Nanchong, Sichuan, China. (Grant numbers 19SXHZ0148)

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Author notes

  1. Anji Xiong, Hongxu Cui and Ruiting Deng have contributed equally to this work and share first authorship.

Authors and Affiliations

  1. Department of Rheumatology and Immunology, Nanchong Central Hospital, The Affiliated Nanchong Central Hospital of North Sichuan Medical College, Nanchong, Sichuan, China

    Anji Xiong, Hongxu Cui & Ruiting Deng

  2. Inflammation and Immunology Key Laboratory of Nanchong, Nanchong, Sichuan, China

    Anji Xiong

  3. Department of Ophthalmology, West China Hospital, Sichuan University, Chengdu, China

    Xin Wei

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  1. Anji Xiong
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  2. Hongxu Cui
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Correspondence to Anji Xiong or Xin Wei.

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Xiong, A., Cui, H., Deng, R. et al. Cyclophosphamide in the Treatment of Systemic Lupus Erythematosus-related Guillain-Barré Syndrome: A Systematic Review of Case Reports. J Neuroimmune Pharmacol 18, 285–293 (2023). https://doi.org/10.1007/s11481-023-10075-w

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