Fibromuscular Dysplasia and Spontaneous Cervical Artery Dissection.

Importance: Fibromuscular dysplasia (FMD) is found in 6 to 14% of patients with spontaneous cervical artery dissection (SCEAD) and may be associated with recurrent SCEAD. Objective: To evaluate the correlates of FMD in patients with SCEAD and to determine whether FMD is associated with recurrent SCEAD. Design, Setting, and Participants: This cohort study included patients from the Stroke Prevention in Cervical Artery Dissection (STOP-CAD) retrospective cohort study who presented from January 2015 to December 2022. This multicenter and international cohort included consecutive adult patients presenting at acute care hospitals and diagnosed with SCEAD in 63 sites in 16 countries. Data were analyzed from April to November 2024. Exposure: Fibromuscular dysplasia was defined as either a history of FMD or presence of FMD on cervical or renal artery imaging. Main Outcomes and Measures: Clinical and radiological correlates were compared between patients with and without FMD using logistic regression models. Rates of recurrent SCEAD by 24 months were compared using a Cox proportional hazards model. Results: This study included 3714 patients with SCEAD (median [IQR] age, 47 [38-56] years; 1637 [44.1%] females), of whom 196 (5.3%) had FMD. Patients with FMD were older (aOR per 10 years, 1.28; 95% CI, 1.14-1.43) and more often female (aOR, 2.00; 95% CI, 1.45-2.75). They more often had a history of dissection involving a noncervical artery (aOR, 8.10; 95% CI, 2.64-24.83), a history of SCEAD (aOR, 2.05; 95% CI, 1.07-3.93), a recent upper respiratory tract infection (aOR, 2.40; 95% CI, 1.52-3.78), a cerebral aneurysm (aOR, 2.22; 95% CI, 1.22-4.06), or a history of migraines (aOR, 2.44; 95% CI, 1.75-3.40). On imaging, they were less likely to have a single vertebral artery dissection (aOR, 0.37; 95% CI, 0.25-0.55) or an occlusive dissection (aOR, 0.55; 95% CI, 0.38-0.78). Eighty-one patients experienced a recurrent SCEAD, of which 46 (56.8%) occurred in the first 3 months of follow-up. The 24-month risk of recurrent SCEAD was 7.7% (95% CI, 3.1%-12.2%) and 2.8% (95% CI, 2.1%-3.5%) in patients with and without FMD, respectively (aHR, 2.75; 95% CI, 1.46-5.18; P = .002). Conclusions and relevance: In this cohort study of patients with SCEAD, FMD was associated with distinct correlates and a higher rate of recurrent SCEAD. These findings may help physicians in identifying and counseling patients with FMD and SCEAD.