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

Introduction/aims: Outcomes in chronic inflammatory demyelinating polyneuropathy (CIDP) have been reported in longitudinal and cross-sectional studies. A considerable variation in long-term disease outcome has appeared in those reports. To overcome this uncertainty, a systematic review and meta-analysis was conducted on CIDP outcomes, including the parameters of case fatality rate, ambulation, physical ability, and remission.

Methods: In accordance with the Preferred Reporting Items for Systematic Reviews and Meta-Analyses guidelines, a systematic search was conducted in PubMed and EMBASE (OVID) for reports with at least 2 years of follow-up on patients with active or previously active CIDP that were published no later than May 12, 2022. Studies were appraised for quality using the Joanna Briggs Institute Critical Appraisal Checklist for studies reporting prevalence data. Pooled analyses were conducted and the results were visualized using forest plots. The study protocol was registered prospectively on PROSPERO (CRD42021266903).

Results: A total of 1290 titles were identified. Sixty-nine full-text articles were screened and 21 studies with 1199 patients were selected for the data analysis. The pooled case fatality rate was 3.3% (95% confidence interval [CI], 1.9% to 5.7%). The pooled fraction of nonambulatory patients was 8.2% (95% CI, 5.7% to 11.6%) and, overall, 47.1% (95% CI, 39.5% to 54.9%) of CIDP patients had a good outcome without disability. The pooled rate of remission was 40.8% (95% CI, 30.6% to 51.8%).

Discussion: Future research is warranted on how to prevent long-term impairment in CIDP. Care should be taken in developing clinical strategies to avoid immunomodulating therapy in the many patients in remission.