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Meta-Analysis
. 2019;52(3-4):161-172.
doi: 10.1159/000494291. Epub 2019 Jan 22.

Incidence and Prevalence of Chronic Inflammatory Demyelinating Polyradiculoneuropathy: A Systematic Review and Meta-Analysis

Merel C Broers  1 , Carina Bunschoten  2 , Daan Nieboer  3 , Hester F Lingsma  3 , Bart C Jacobs  2   4
Affiliations
Meta-Analysis

Incidence and Prevalence of Chronic Inflammatory Demyelinating Polyradiculoneuropathy: A Systematic Review and Meta-Analysis

Merel C Broers et al. Neuroepidemiology. 2019.

Abstract

Background: Prevalence and incidence rates of chronic inflammatory demyelinating polyradiculoneuropathy (CIDP) are required to determine the impact of CIDP on society. We aimed to estimate the prevalence and incidence of CIDP worldwide and to determine the effect of diagnostic criteria on prevalence and incidence.

Method: A systematic review was conducted for all published incidence and prevalence studies on CIDP until May 18, 2017. Methodological quality was assessed using the Methodological Evaluation of Observational Research checklist. We performed a random effect meta-analysis to estimate pooled prevalence and incidence rates.

Results: Of the 907 studies, 11 were included in the systematic review, 5 in the meta-analysis of incidence (818 cases; 220,513,514 person-years) and 9 in the meta-analysis of prevalence (3,160 cases; 160,765,325 population). These studies had a moderate quality. The pooled crude incidence rate was 0.33 per 100,000 person-years (95% CI 0.21-0.53; I2 = 95.7%) and the pooled prevalence rate was 2.81 per 100,000 (95% CI 1.58-4.39; I2 = 99.1%). Substantial heterogeneity in incidence and prevalence across studies seems to be partly explained by using different diagnostic criteria.

Conclusion: These findings provide a starting point to estimate the social burden of CIDP and demonstrate the need to reach consensus on diagnostic criteria for CIDP.

Keywords: Chronic inflammatory demyelinating polyradiculoneuropathy; Incidence; Meta-analysis; Prevalence; Systematic review.

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Figures

Fig. 1
Fig. 1
Preferred Reporting Items for Systematic Reviews and Meta-Analyses (PRISMA) flowchart of the study selection process.
Fig. 2
Fig. 2
Pooled crude incidence rate using the random-effects model. RE model, random-effects model.
Fig. 3
Fig. 3
Pooled crude prevalence rate using the random-effects model. RE model, random-effects model.
See this image and copyright information in PMC

References

    1. Vallat JM, Sommer C, Magy L. Chronic inflammatory demyelinating polyradiculoneuropathy: diagnostic and therapeutic challenges for a treatable condition. Lancet Neurol. 2010;9:402–412. - PubMed
    1. Nobile-Orazio E. Chronic inflammatory ­demyelinating polyradiculoneuropathy and variants: where we are and where we should go. J Peripher Nerv Syst. 2014;19:2–13. - PubMed
    1. Koller H, Kieseier BC, Jander S, Hartung HP. Chronic inflammatory demyelinating polyneuropathy. N Engl J Med. 2005;352:1343–1356. - PubMed
    1. Joint Task. Force of the EFNS and the PNS European Federation of Neurological Societies/Peripheral Nerve Society Guideline on management of chronic inflammatory demyelinating polyradiculoneuropathy: report of a joint task force of the European Federation of Neurological Societies and the Peripheral Nerve Society - First Revision. J Peripher Nerv Syst. 2010;15:1–9. - PubMed
    1. Hughes R, Bensa S, Willison H, Van den Bergh P, Comi G, Illa I, Nobile-Orazio E, van Doorn P, Dalakas M, Bojar M, Swan A; Inflammatory Neuropathy Cause and Treatment (INCAT) Group Randomized controlled trial of intravenous immunoglobulin versus oral prednisolone in chronic inflammatory demyelinating polyradiculoneuropathy. Ann Neurol. 2001;50:195–201. - PubMed

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