{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,26]],"date-time":"2026-09-26T06:00:06Z","timestamp":1790402406912,"version":"4.1.0"},"reference-count":34,"publisher":"Wiley","license":[{"start":{"date-parts":[[2026,9,25]],"date-time":"2026-09-25T00:00:00Z","timestamp":1790294400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/onlinelibrary.wiley.com\/termsAndConditions#vor"},{"start":{"date-parts":[[2026,9,25]],"date-time":"2026-09-25T00:00:00Z","timestamp":1790294400000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["J Intern Med"],"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:p>Vascular Ehlers\u2013Danlos syndrome (vEDS), caused by likely pathogenic COL3A1 variants, carries a high risk of arterial dissection or rupture, intestinal perforation and uterine rupture, and a median survival near 51 years. It is often recognized only at a catastrophe, and its acute management is deliberately counter\u2010intuitive. The therapeutic landscape changed in 2025 with the first randomized evidence for an angiotensin\u2010receptor blocker. We provide a clinical, practice\u2010oriented review for internists and the multidisciplinary team. As is appropriate for a rare disease, case\u2010level evidence was considered alongside cohorts and trials. Suspicion is the rate\u2010limiting step, and we set out speciality\u2010specific red flags. Diagnosis requires molecular confirmation, with differentiation from other heritable arteriopathies. Genotype is a probabilistic predisposition, not a deterministic predictor: there is ascertainment bias, reduced penetrance and intrafamilial variability temper inference. Emergency care is harm\u2010avoidant\u2014catheter angiography, elective endoscopy and unnecessary surgery should be avoided, and endovascular and tissue\u2010sparing techniques preferred. Pharmacological prevention rests on celiprolol, with randomized evidence now supporting irbesartan added to background celiprolol, and expert\u2010centred consideration of angiotensin\u2010receptor blockade when celiprolol is not tolerated. Surveillance should be individualized along the European Reference Network on Rare Multisystemic Vascular Diseases logic. Pregnancy, psychosocial care and cascade testing complete lifelong management. In vEDS, earlier recognition and the avoidance of iatrogenic harm\u2014not new molecules\u2014offer the largest near\u2010term gains in survival and quality of life.<\/jats:p>","DOI":"10.1111\/joim.70165","type":"journal-article","created":{"date-parts":[[2026,9,26]],"date-time":"2026-09-26T05:40:26Z","timestamp":1790401226000},"update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":0,"title":["Vascular Ehlers\u2013Danlos syndrome: A multidisciplinary clinical framework for early suspicion, emergency\u2010safe management and lifelong care"],"prefix":"10.1111","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-5621-1374","authenticated-orcid":false,"given":"Salvatore","family":"Corrao","sequence":"first","affiliation":[{"name":"Department of Clinical Medicine Internal Medicine Unit (with Rheumatology, Dermatology, Diabetology and Tertiary Diabetic\u2010Foot Care) ARNAS Civico, Di Cristina, Benfratelli  Palermo Italy"},{"name":"Department of Health Promotion Sciences Maternal and Infant Care Internal Medicine and Medical Specialties (PROMISE) University of Palermo  Palermo Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maria Irene","family":"Ruggeri","sequence":"additional","affiliation":[{"name":"Department of Clinical Medicine Internal Medicine Unit (with Rheumatology, Dermatology, Diabetology and Tertiary Diabetic\u2010Foot Care) ARNAS Civico, Di Cristina, Benfratelli  Palermo Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Antonino Di","family":"Benedetto","sequence":"additional","affiliation":[{"name":"Department of Clinical Medicine Internal Medicine Unit (with Rheumatology, Dermatology, Diabetology and Tertiary Diabetic\u2010Foot Care) ARNAS Civico, Di Cristina, Benfratelli  Palermo Italy"},{"name":"Department of Health Promotion Sciences Maternal and Infant Care Internal Medicine and Medical Specialties (PROMISE) University of Palermo  Palermo Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0006-0907-5086","authenticated-orcid":false,"given":"Giacomo","family":"Corrao","sequence":"additional","affiliation":[{"name":"Department of Internal Medicine Azienda Ospedaliera Universitaria \u201cPoliclinico G. Martino\u201d University of Messina  Messina Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0009-2702-5551","authenticated-orcid":false,"given":"Luigi","family":"Calvo","sequence":"additional","affiliation":[{"name":"Department of Clinical Medicine Internal Medicine Unit (with Rheumatology, Dermatology, Diabetology and Tertiary Diabetic\u2010Foot Care) ARNAS Civico, Di Cristina, Benfratelli  Palermo Italy"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0009-0003-3236-3340","authenticated-orcid":false,"given":"Salvatore","family":"Scibetta","sequence":"additional","affiliation":[{"name":"Department of Clinical Medicine Internal Medicine Unit (with Rheumatology, Dermatology, Diabetology and Tertiary Diabetic\u2010Foot Care) ARNAS Civico, Di Cristina, Benfratelli  Palermo Italy"},{"name":"Institute for Biomedical Research and Innovation (IRIB) National Research Council (CNR)  Palermo 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