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Background <p>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.</p> Methods <p>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.</p> Results <p>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.</p> Conclusions <p>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.</p>

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Prevalence of vascular complications in Ehlers-Danlos syndrome: a systematic review and meta-analysis

  • 期刊论文

  • 出版模式：

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  • 发表日期：

    2025年6月20日

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  • Abdelaziz A. Awad,
  • Ambana Yappalparvi,
  • Mahalaqua Nazli Khatib,
  • Roopashree R,
  • Mandeep Kaur,
  • Manish Srivastava,
  • Amit Barwal,
  • G. V. Siva Prasad,
  • Pranchal Rajput,
  • Rukshar Syed,
  • Gajendra Sharma,
  • Anand Prasoon,
  • Muhammed Shabil,
  • Ankit Punia,
  • Megha Jagga,
  • Rachana Mehta,
  • Sanjit Sah,
  • Prakasini Satapathy,
  • Abhay M Gaidhane,
  • Edward Mawejje,
  • Ganesh Bushi

摘要

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.