Cervical artery lesions are ‘frequent and mostly asymptomatic’ in vascular Ehlers-Danlos syndrome patients

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Cervical artery lesions are frequent and mostly asymptomatic in patients with vascular Ehlers-Danlos syndrome, investigators seeking to systematically assess arterial complications in the region found.

“Local dissections and aneurysms are the most frequent type of lesions, but transient ischemic attack or stroke seem rare,” the France-based research team concluded in a paper entitled “Spontaneous cervical artery dissection in vascular Ehlers-Danlos syndrome,” recently published in Stroke.

The researchers, led by Salma Adham, MD, of Centre de Référence des Maladies Vasculaires Rares, Hôpital Européen Georges Pompidou, in Paris set out to establish the first systematic assessment of cervical artery lesions.

“Vascular Ehlers-Danlos syndrome is a rare inherited connective tissue disorder because of pathogenic variants in the COL3A1 gene,” Adham et al write. “Arterial complications can affect all anatomic areas and about 25% involve supra-aortic trunks (SATs) but no systematic assessment of cervical artery lesions has been made.”

They carried out a retrospective review of patients with molecularly proven vascular Ehlers-Danlos syndrome at a tertiary referral center from 2000–2017.

Of 144 patients in the cohort analyzed, 56.9% (n=82) had SAT lesions: 64.6% female and 74.4% index-case patients. Most lesions were identified in early arterial assessment—48% at first work-up, the investigators found.

“Cumulative incidence of a first identification of a SAT lesion was 41.7% at 40 years old,” the note. “On the complete period of survey, 183 SAT lesions (with 132 dissections and 33 aneurysms) were identified, mainly in internal carotid arteries (56.3%) and vertebral arteries (28.9%), more rarely in patients with COL3A1 null mutations (p=0.008). Transient ischemic attack or stroke were reported in n=16 (19.5%) of the 82 patients with SAT lesions without relation with age, sex, treatment, or hypertension.”

SOURCE: doi.org/10.1161/STROKEAHA.120.032106

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