SynthesisOrphanet journal of rare diseases2025
Prevalence of vascular complications in Ehlers-Danlos syndrome: a systematic review and meta-analysis.
Synthesis in Orphanet journal of rare diseases, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 4 papers.
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Who cites it
4 citing papers in PubMed.
- A rare case report of vascular Ehlers-Danlos syndrome as a pulsatile neck mass with an internal carotid artery aneurysm.Radiology case reports · 2026Article
- Association between ocular manifestations and incident cardiovascular comorbidities in Ehlers Danlos Syndrome.Eye (London, England) · 2026Article
- Vascular Phenotype of Kyphoscoliotic Ehlers-Danlos Syndrome.Indian journal of pediatrics · 2026Article
- Clinically Translatable Mutation-Based Biomarkers in Ascending Aortic Aneurysm: A Bibliometric Study.Human mutation · 2026Article
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Authors and funding
21 authors.
Funding
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Abstract
backgroundEhlers-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.
methodsThe 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.
resultsOf 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.
conclusionsThis 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.
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