Extra-cervical arterial involvement in patients with spontaneous cervical artery dissection

BACKGROUND: The magnitude of the association between fibromuscular dysplasia (FMD) and spontaneous cervical artery dissection (sCeAD) remains uncertain, since data available derive from uncompleted vascular screening. This study aims to assess the frequency and types of cervical and extra-cervical arterial lesions, particularly of the FMD type, in patients with sCeAD. METHODS: We recruited all patients with a diagnosis of sCeAD from January 2016 to December 2023. All patients underwent full-body vascular imaging to assess the presence of cervical and extra-cervical multifocal and focal stenosis, aneurysms, dissections, tortuosity, ectasia/dilatation or parietal irregularities. RESULTS: Of the 94 patients included (65.7% male; 48.8 ± 8.7 years old), 31.9% had evidence of cerebrovascular FMD. After a whole-body vascular screening, all-type of extra-cervical vascular abnormalities were identified in 47.5% of patients. Twenty-one percent of patients (21.3%) had evidence of extra-cervical FMD, affecting renal (11.5%), visceral (13.1%), and limb (6.7%) arteries. Prevalence of extra-cervical dissections and aneurysms was 14.8% and 9.8%, respectively. After a whole-body screening, overall prevalence of FMD raised from 32.8% to 39.3%. Patients with FMD were mainly female ( P < 0.0001), had a history of migraine ( P = 0.023) and recurrent sCeADs ( P = 0.025). After a multivariate analysis, female sex ( P < 0.0001) and, almost, a positive history of recurrent sCeADs ( P = 0.053) were identified as predictors of FMD in patients with sCeADs. CONCLUSIONS: The study reveals a high prevalence of FMD and other vascular abnormalities outside the cervical arteries in patients with sCeAD, highlighting the importance of a comprehensive vascular screening, especially in women and in patients with a positive history of recurrent sCeAD.