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Cyclophosphamide for Treatment of Refractory Chronic Inflammatory Demyelinating Polyradiculoneuropathy: A Systematic Review and Meta-analysis

  • Author Footnotes
    # These authors are co–primary authors.
    Qilang Xiang
    Footnotes
    # These authors are co–primary authors.
    Affiliations
    Department of Rheumatology and Immunology, Nanchong Central Hospital, The Affiliated Nanchong Central Hospital of North Sichuan Medical College, Nanchong, China
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  • Author Footnotes
    # These authors are co–primary authors.
    Yuzi Cao
    Footnotes
    # These authors are co–primary authors.
    Affiliations
    Department of Rheumatology and Immunology, Nanchong Central Hospital, The Affiliated Nanchong Central Hospital of North Sichuan Medical College, Nanchong, China
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  • Author Footnotes
    # These authors are co–primary authors.
    Zhuoyao Song
    Footnotes
    # These authors are co–primary authors.
    Affiliations
    Department of Rheumatology and Immunology, Nanchong Central Hospital, The Affiliated Nanchong Central Hospital of North Sichuan Medical College, Nanchong, China
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  • Huini Chen
    Affiliations
    Department of Rheumatology and Immunology, Nanchong Central Hospital, The Affiliated Nanchong Central Hospital of North Sichuan Medical College, Nanchong, China
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  • Ziyi Hu
    Affiliations
    Department of Rheumatology and Immunology, Nanchong Central Hospital, The Affiliated Nanchong Central Hospital of North Sichuan Medical College, Nanchong, China
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  • Shifeng Zhou
    Affiliations
    Department of Rheumatology and Immunology, Nanchong Central Hospital, The Affiliated Nanchong Central Hospital of North Sichuan Medical College, Nanchong, China
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  • Yan Zhang
    Affiliations
    Department of Rheumatology and Immunology, Nanchong Central Hospital, The Affiliated Nanchong Central Hospital of North Sichuan Medical College, Nanchong, China
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  • Hongxu Cui
    Affiliations
    Department of Rheumatology and Immunology, Nanchong Central Hospital, The Affiliated Nanchong Central Hospital of North Sichuan Medical College, Nanchong, China
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  • Jie Luo
    Affiliations
    Department of Rheumatology and Immunology, Nanchong Central Hospital, The Affiliated Nanchong Central Hospital of North Sichuan Medical College, Nanchong, China
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  • Yiying Qiang
    Affiliations
    Department of Rheumatology and Immunology, Nanchong Central Hospital, The Affiliated Nanchong Central Hospital of North Sichuan Medical College, Nanchong, China
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  • Ye Wang
    Affiliations
    Department of Rheumatology and Immunology, Nanchong Central Hospital, The Affiliated Nanchong Central Hospital of North Sichuan Medical College, Nanchong, China
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  • Shiquan Shuai
    Affiliations
    Department of Rheumatology and Immunology, Nanchong Central Hospital, The Affiliated Nanchong Central Hospital of North Sichuan Medical College, Nanchong, China

    Inflammation and Immunology Key Laboratory of Nanchong City, Sichuan, China
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  • Yuan Yang
    Affiliations
    Department of Rheumatology and Immunology, The First Affiliated Hospital of Chengdu Medical College, Chengdu, China
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  • Min Yang
    Affiliations
    Department of Rheumatology and Immunology, West China Hospital, Sichuan University, Chengdu, China
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  • Author Footnotes
    † These authors contributed equally to this work.
    Xin Wei
    Footnotes
    † These authors contributed equally to this work.
    Affiliations
    Department of Ophthalmology, West China Hospital, Sichuan University; Chengdu, China
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  • Author Footnotes
    † These authors contributed equally to this work.
    Anji Xiong
    Correspondence
    Address correspondence to: Anji Xiong, Department of Rheumatology and Immunology, Nanchong Central Hospital, The Affiliated Nanchong Central Hospital of North Sichuan Medical College, No. 97, Renmin Nan Lu, Nanchong, Sichuan, China.
    Footnotes
    † These authors contributed equally to this work.
    Affiliations
    Department of Rheumatology and Immunology, Nanchong Central Hospital, The Affiliated Nanchong Central Hospital of North Sichuan Medical College, Nanchong, China

    Inflammation and Immunology Key Laboratory of Nanchong City, Sichuan, China
    Search for articles by this author
  • Author Footnotes
    # These authors are co–primary authors.
    † These authors contributed equally to this work.

      Abstract

      Purpose

      This study evaluates the tolerability and efficacy of cyclophosphamide (CYC) for the treatment of refractory chronic inflammatory demyelinating polyradiculoneuropathy (CIDP).

      Methods

      We searched PubMed, Embase, Cochrane Library, and 2 Chinese databases (Chinese National Knowledge Infrastructure and SinoMed) for studies published between database inception and September 30, 2021. Articles obtained using the appropriate keywords were independently selected by 2 reviewers on the basis of the established inclusion and exclusion criteria.

      Findings

      In total, 240 records that were eventually curtailed to 13 studies with 83 patients were retrieved and subsequently included in this evaluation. All 13 studies were included in the systematic review, 7 of which were included in the meta-analysis. The pooled estimate of the response rate was 68% (95% CI, 45%–90%). The pooled estimate of the adverse reaction rate was 8% (95% CI, 0%–15%). The disease duration before the first CYC treatment was negatively correlated with the reduction in the modified Rankin Scale score (r = −0.76, P < 0.001). However, the response rates did not differ significantly between patients of different sexes (P = 0.716) or between patients who received and those who did not receive concurrent glucocorticoids (P = 0.617).

      Implications

      CYC might be a recommended therapeutic option for patients with refractory CIDP, especially those who are unable to accept rituximab treatment. Earlier CYC treatment yields better therapeutic outcomes in patients with refractory CIDP without CYC-related contraindications.

      Graphical abstract

      Key words

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