Review

. 2025 Jun 20;20(1):312.

doi: 10.1186/s13023-025-03854-6.

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

Abdelaziz A Awad^#¹, Ambana Yappalparvi^#², Mahalaqua Nazli Khatib^#³, Roopashree R⁴, Mandeep Kaur⁵, Manish Srivastava⁶, Amit Barwal⁷, G V Siva Prasad⁸, Pranchal Rajput⁹, Rukshar Syed¹⁰, Gajendra Sharma¹¹, Anand Prasoon¹², Muhammed Shabil^{13

14}, Ankit Punia¹⁵, Megha Jagga¹⁶, Rachana Mehta¹⁷, Sanjit Sah^{18

19}, Prakasini Satapathy^#^{20

21}, Abhay M Gaidhane²², Edward Mawejje²³, Ganesh Bushi²⁴

Affiliations

¹ Faculty of Medicine, Al-Azhar university, Cairo, Egypt. awad.abdelaziz.0505@gmail.com.
² Noida Institute of Engineering and Technology (Pharmacy Institute), Greater, Noida, India.
³ Division of Evidence Synthesis, Global Consortium of Public Health and Research, Datta Meghe Institute of Higher Education, Wardha, India.
⁴ Department of Chemistry and Biochemistry, School of Sciences, JAIN (Deemed to be University), Bangalore, Karnataka, India.
⁵ Department of Allied Healthcare and Sciences, Vivekananda Global University, Jaipur, Rajasthan, 303012, India.
⁶ Department of Endocrinology, NIMS University, Jaipur, India.
⁷ Chandigarh Pharmacy College, Chandigarh Group of College, Mohali - 140307, Jhanjeri, Punjab, India.
⁸ Department of Chemistry, Raghu Engineering College, Visakhapatnam, 531162, Andhra Pradesh, India.
⁹ School of Applied and Life Sciences, Division of Research and Innovation, Uttaranchal University, Dehradun, India.
¹⁰ IES Institute of Pharmacy, IES University, Bhopal, 462044, Madhya Pradesh, India.
¹¹ New Delhi Institute of Management, Tughlakabad Institutional Area, New Delhi, India.
¹² Graphic Era Institute of Medical Sciences, Graphic Era (Deemed to be University, Clement Town, Dehradun, India.
¹³ University Center for Research and Development, Chandigarh University, Mohali, Punjab, India.
¹⁴ Medical Laboratories Techniques Department, AL-Mustaqbal University, 51001 Hillah, Babil, Iraq.
¹⁵ Centre of Research Impact and Outcome, Chitkara University, Rajpura, 140417, Punjab, India.
¹⁶ Chitkara Centre for Research and Development, Chitkara University, Himachal Pradesh, 174103, India.
¹⁷ Clinical Microbiology, RDC, Manav Rachna International Institute of Research and Studies, Faridabad, 121004, Haryana, India.
¹⁸ Department of Paediatrics, Research Centre, Dr. D. Y. Patil Medical College Hospital, Dr. D. Y. Patil Vidyapeeth (Deemed-to-be-University), Pimpri, Pune, 411018, Maharashtra, India.
¹⁹ Department of Medicine, Korea Universtiy, Seoul, South Korea.
²⁰ Center for Global Health Research, Saveetha Institute of Medical and Technical Sciences, Saveetha Medical College and Hospital, Saveetha University, Chennai, India.
²¹ Faculty of Data Science and Information Technology, INTI International University, Nilai, Malaysia.
²² Global Health Academy, School of Epidemiology and Public Health, Jawaharlal Nehru Medical College, Datta Meghe Institute of Higher Education, Wardha, India. abhay.psm@dmiher.edu.in.
²³ School of Public Health, Makerere University College of Health Sciences, Mulago Hill, Kampala, Uganda.
²⁴ School of Pharmaceutical Sciences, Lovely Professional University, Phagwara, India.

^# Contributed equally.

PMID: 40542421
PMCID: PMC12180213
DOI: 10.1186/s13023-025-03854-6

Review

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

Abdelaziz A Awad et al. Orphanet J Rare Dis. 2025.

. 2025 Jun 20;20(1):312.

doi: 10.1186/s13023-025-03854-6.

Authors

Affiliations

¹ Faculty of Medicine, Al-Azhar university, Cairo, Egypt. awad.abdelaziz.0505@gmail.com.
² Noida Institute of Engineering and Technology (Pharmacy Institute), Greater, Noida, India.
³ Division of Evidence Synthesis, Global Consortium of Public Health and Research, Datta Meghe Institute of Higher Education, Wardha, India.
⁴ Department of Chemistry and Biochemistry, School of Sciences, JAIN (Deemed to be University), Bangalore, Karnataka, India.
⁵ Department of Allied Healthcare and Sciences, Vivekananda Global University, Jaipur, Rajasthan, 303012, India.
⁶ Department of Endocrinology, NIMS University, Jaipur, India.
⁷ Chandigarh Pharmacy College, Chandigarh Group of College, Mohali - 140307, Jhanjeri, Punjab, India.
⁸ Department of Chemistry, Raghu Engineering College, Visakhapatnam, 531162, Andhra Pradesh, India.
⁹ School of Applied and Life Sciences, Division of Research and Innovation, Uttaranchal University, Dehradun, India.
¹⁰ IES Institute of Pharmacy, IES University, Bhopal, 462044, Madhya Pradesh, India.
¹¹ New Delhi Institute of Management, Tughlakabad Institutional Area, New Delhi, India.
¹² Graphic Era Institute of Medical Sciences, Graphic Era (Deemed to be University, Clement Town, Dehradun, India.
¹³ University Center for Research and Development, Chandigarh University, Mohali, Punjab, India.
¹⁴ Medical Laboratories Techniques Department, AL-Mustaqbal University, 51001 Hillah, Babil, Iraq.
¹⁵ Centre of Research Impact and Outcome, Chitkara University, Rajpura, 140417, Punjab, India.
¹⁶ Chitkara Centre for Research and Development, Chitkara University, Himachal Pradesh, 174103, India.
¹⁷ Clinical Microbiology, RDC, Manav Rachna International Institute of Research and Studies, Faridabad, 121004, Haryana, India.
¹⁸ Department of Paediatrics, Research Centre, Dr. D. Y. Patil Medical College Hospital, Dr. D. Y. Patil Vidyapeeth (Deemed-to-be-University), Pimpri, Pune, 411018, Maharashtra, India.
¹⁹ Department of Medicine, Korea Universtiy, Seoul, South Korea.
²⁰ Center for Global Health Research, Saveetha Institute of Medical and Technical Sciences, Saveetha Medical College and Hospital, Saveetha University, Chennai, India.
²¹ Faculty of Data Science and Information Technology, INTI International University, Nilai, Malaysia.
²² Global Health Academy, School of Epidemiology and Public Health, Jawaharlal Nehru Medical College, Datta Meghe Institute of Higher Education, Wardha, India. abhay.psm@dmiher.edu.in.
²³ School of Public Health, Makerere University College of Health Sciences, Mulago Hill, Kampala, Uganda.
²⁴ School of Pharmaceutical Sciences, Lovely Professional University, Phagwara, India.

^# Contributed equally.

PMID: 40542421
PMCID: PMC12180213
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.

Keywords: Ehlers-Danlos syndrome; Meta-analysis; Prevalence; Vascular complications.

PubMed Disclaimer

Conflict of interest statement

Declarations. Ethics approval and consent to participate: Not applicable, as there were no human participants involved in this study. Consent for publication: Not applicable, as this study does not involve any individual person’s data in any form. Competing interests: The authors report no conflict of interest. Consent to participate: Not applicable, as there were no human participants involved in this study.

Figures

**Fig. 1**
PRISMA flowchart showing the selection process of included studies

**Fig. 2**
Forest plot presenting the prevalence of vascular complications in EDS

**Fig. 3**
Sensitivity analysis plot illustrating vascular complications in EDS

**Fig. 4**
Doi plot assessing publication bias in studies on vascular complications in EDS

See this image and copyright information in PMC

References

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

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

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