Orphanet J Rare Dis. 2025 Jun 20;20(1):312. doi: 10.1186/s13023-025-03854-6.
ABSTRACT
BACKGROUND: Ehlers-Danlos Syndrome (EDS) comprises connective tissue disorders associated with increased vascular complication risks. This meta-analysis assesses the prevalence of vascular complications in among patients with EDS.
METHODS: The review was conducted following PRISMA guidelines. A comprehensive literature search was conducted in PubMed, Embase, and Web of Science until November 2024. Observational studies reporting vascular complications in EDS were included. Data extraction included demographics, complication types, and study design, and quality assessment was evaluated using the modified Newcastle-Ottawa Scale (NOS). Random-effects models and I² statistics assessed heterogeneity, while Doi plots evaluated publication bias.
RESULTS: Of the 1,772 articles screened, 12 met the inclusion criteria, reporting various vascular complications in EDS. The overall pooled prevalence of vascular complications was 30.03% (95% CI: 15.00-51.07%). The prevalence for the vEDS subtype was 42.36% (95% CI: 12.63-78.88%), for unspecified EDS was 18.65% (95% CI: 5.38-48.03%), and for hEDS was 19.77% (95% CI: 15.09-25.16%). Sensitivity analyses confirmed the stability of the pooled prevalence estimates, and DOI plots indicated minimal publication bias.
CONCLUSIONS: This review highlights the high risk of vascular complications in vEDS, with moderate involvement in other EDS subtypes. Regular vascular monitoring, especially in vEDS, is crucial for early detection and intervention. Standardized diagnostic protocols and further research into genetic factors are needed to improve management strategies.
PMID:40542421 | DOI:10.1186/s13023-025-03854-6
