Challenges in the management of patients with vascular Ehlers-Danlos syndrome: lessons from three clinical cases

Vascular Ehlers-Danlos syndrome (vEDS) is a rare inherited connective tissue disorder marked by extreme arterial fragility, requiring high clinical suspicion to avoid misdiagnosis and inappropriate interventions.Strict adherence to evidence-based pharmacological therapy and structured long-term follow-up are crucial, as treatment discontinuation may lead to rapid and life-threatening vascular events.Management should be centralized in expert centres with a multidisciplinary approach, where individualized and predominantly conservative strategies can optimize outcomes in this high-risk population.

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Publication Details

Journal
European Journal of Case Reports in Internal Medicine
Published
2025-12-04
DOI
https://doi.org/10.12890/2025_005837
Primary Topic
Connective tissue disorders research
Type
article
Field-Weighted Citation Impact
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article

Challenges in the management of patients with vascular Ehlers-Danlos syndrome: lessons from three clinical cases

Tarek Sulaiman, Giacomo Buso, Matteo Lemoli, Adriana J. Skafi et al.
European Journal of Case Reports in Internal Medicine
Connective tissue disorders research
article

Challenges in the management of patients with vascular Ehlers-Danlos syndrome: lessons from three clinical cases

Tarek Sulaiman, Giacomo Buso, Matteo Lemoli, Adriana J. Skafi, Maria Lorenza Muiesan, Lara Alayasa, Razan Fiala
article en

Abstract

Vascular Ehlers-Danlos syndrome (vEDS) is a rare inherited connective tissue disorder marked by extreme arterial fragility, requiring high clinical suspicion to avoid misdiagnosis and inappropriate interventions.Strict adherence to evidence-based pharmacological therapy and structured long-term follow-up are crucial, as treatment discontinuation may lead to rapid and life-threatening vascular events.Management should be centralized in expert centres with a multidisciplinary approach, where individualized and predominantly conservative strategies can optimize outcomes in this high-risk population.

European Journal of Case Reports in Internal MedicineVol. 12(12)
Al-Quds University (PS), University of Brescia (IT)
Openalex Percentile: Top 60%
Connective tissue disorders research
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