{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2025,12,5]],"date-time":"2025-12-05T14:12:24Z","timestamp":1764943944299,"version":"3.46.0"},"reference-count":10,"publisher":"SMC Media","issue":"12","content-domain":{"domain":[],"crossmark-restriction":false},"short-container-title":[],"abstract":"<jats:p>Vascular Ehlers-Danlos syndrome (vEDS) is one of the most severe heritable connective tissue disorders, caused by pathogenic variants in\u00a0COL3A1\u00a0and characterized by arterial fragility, premature mortality, and a high burden of vascular events. Management remains particularly challenging, requiring careful balance between the risks of intervention and the prevention of catastrophic complications. We present three cases of genetically confirmed vEDS\u00a0that exemplify the complexity of patient care in this setting. These cases underline the importance of early referral to expert centers, where multidisciplinary care, genetic counseling, and structured follow-up can be guaranteed. They also highlight the central role of therapeutic adherence and long-term surveillance, as interruption of validated medical therapy may lead to life-threatening vascular events. Pharmacological treatment with celiprolol remains the first strategy supported by randomized trial evidence, while recent data suggest that the addition of angiotensin receptor blockers may further reduce vascular risk. Nevertheless, outcomes remain poor, and the prognosis of vEDS continues to be dominated by recurrent arterial complications despite optimal management. Conservative strategies are generally preferred, with invasive interventions reserved for life-threatening circumstances and best performed in specialized centers. These cases\u00a0underscore both the progress made and the unmet need for novel therapeutic options to improve outcomes in this high-risk population.<\/jats:p>","DOI":"10.12890\/2025_005837","type":"journal-article","created":{"date-parts":[[2025,12,4]],"date-time":"2025-12-04T15:06:49Z","timestamp":1764860809000},"source":"Crossref","is-referenced-by-count":0,"title":["Challenges in the management of patients with vascular Ehlers-Danlos syndrome: lessons from three clinical cases"],"prefix":"10.12890","volume":"12","author":[{"given":"Lara","family":"Alayasa","sequence":"first","affiliation":[{"name":"Al-Quds University, Faculty of Medicine, Abu Dis, Palestine"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Razan","family":"Fiala","sequence":"first","affiliation":[{"name":"Al-Quds University, Faculty of Medicine, Abu Dis, Palestine"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Adriana","family":"Skafi","sequence":"first","affiliation":[{"name":"Al-Quds University, Faculty of Medicine, Abu Dis, Palestine"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tala","family":"Sulaiman","sequence":"first","affiliation":[{"name":"Al-Quds University, Faculty of Medicine, Abu Dis, Palestine"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Matteo","family":"Lemoli","sequence":"first","affiliation":[{"name":"Department of Clinical and Experimental Sciences, Division of Internal Medicine, ASST Spedali Civili Brescia, University of Brescia, Brescia, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Giacomo","family":"Buso","sequence":"first","affiliation":[{"name":"Department of Clinical and Experimental Sciences, Division of Internal Medicine, ASST Spedali Civili Brescia, University of Brescia, Brescia, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Maria Lorenza","family":"Muiesan","sequence":"first","affiliation":[{"name":"Department of Clinical and Experimental Sciences, Division of Internal Medicine, ASST Spedali Civili Brescia, University of Brescia, Brescia, Italy"}],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"10397","published-online":{"date-parts":[[2025,12,4]]},"reference":[{"key":"10.12890\/2025_005837_ref1","doi-asserted-by":"crossref","unstructured":"Pepin M, Schwarze U, Superti-Furga A, Byers PH. 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