BMJ Case Rep. 2026 Sep 11;19(9):e274392. doi: 10.1136/bcr-2026-274392.
ABSTRACT
Vascular Ehlers-Danlos syndrome (vEDS) is a rare connective tissue disorder characterised by vascular fragility and risk of arterial complications. Further overlap with autoimmune diseases and multisystem comorbidities creates unique medical management challenges.We present an early 60s female with vEDS suffering from extensive comorbidities including systemic lupus erythematosus, rheumatoid arthritis, fibromuscular dysplasia and more. Over 3 years as our patient, she required multiple surgical interventions (eg, hysterectomy, salpingo-oophorectomy) and multidisciplinary management for chronic pain, autoimmune comorbidities and connective tissue fragility. Unlike more severe presentations commonly reported in vEDS cases, this patient has survived into her 60s and illustrates the spectrum of disease severity.This case highlights the importance of a multidisciplinary approach to complex vEDS, particularly with numerous autoimmune and oncologic comorbidities. While medical decisions must always be carefully individualised, this case illustrates the challenges of coordinating care for an older patient with vEDS and multiple coexisting medical conditions.
PMID:42728080 | DOI:10.1136/bcr-2026-274392