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
Graphical Abstract
Key words
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Article info
Publication history
Published online: July 21, 2022
Accepted:
June 26,
2022
Identification
Copyright
© 2022 Elsevier Inc.
