Outcome in chronic inflammatory demyelinating polyneuropathy: A systematic review and meta-analysis.

Abstract

The outcome in chronic inflammatory demyelinating polyneuropathy (CIDP) has been reported in longitudinal and cross-sectional studies. A considerable variation of long-term disease outcome appears in the reports. To overcome this uncertainty, a systematic review and meta-analyses of CIDP outcome including the parameters case fatality rate, ambulation, physical ability and remission was conducted. In accordance with the PRISMA guidelines, a systematic search in PubMed and EMBASE (OVID) for reports exceeding 2 years of follow-up on patients with active or previously active CIDP published until May 12th , 2022 was conducted. Studies were quality appraised using the Joanna Briggs Institute (JBI) Critical Appraisal Checklist for studies reporting prevalence data. Pooled analyses were conducted and the results were visualized using forest plots. The study protocol was prospectively registered on PROSPERO (CRD42021266903). A total of 1,290 titles were identified. Sixty-nine full-text articles were screened and 21 studies with 1,199 patients were selected for the data analysis. The pooled case fatality rate was 3.3% (95% CI: 1.9% - 5.7%). The pooled fraction of non-ambulatory patients was 8.2% (95% CI: 5.7% - 11.6%) and overall, 47.1% (95% CI: 39.5% - 54.9%) of CIDP patients had a good outcome without disability. In addition, the pooled rate of remission was 40.8% (95% CI: 30.6% - 51.8%). Future research is warranted on how to prevent long-term impairment in CIDP. In addition, care should be taken to develop clinical strategies to avoid immune modulating therapy in the many patients in remission. This article is protected by copyright. All rights reserved.