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Fibromuscular Dysplasia and Spontaneous Cervical Artery Dissection

Ahmad Nehme¹, Liqi Shu², Marion Boulanger¹

¹Neurology, Université Caen-Normandie, CHU de Caen-Normandie, Caen, France.

JAMA Network Open

|November 6, 2025

Abstract

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.

Insights

Fibromuscular dysplasia (FMD) is linked to a higher risk of recurrent spontaneous cervical artery dissection (SCEAD). Identifying FMD in SCEAD patients may aid in risk stratification and patient counseling for better outcomes.

Area of Science:

Vascular Medicine
Neurology
Radiology

Background:

Fibromuscular dysplasia (FMD) affects 6-14% of patients with spontaneous cervical artery dissection (SCEAD).
The association between FMD and recurrent SCEAD requires further investigation.

Purpose of the Study:

To identify clinical and radiological correlates of FMD in patients diagnosed with SCEAD.
To determine if FMD is associated with an increased risk of recurrent SCEAD.

Main Methods:

A retrospective cohort study (STOP-CAD) included 3714 adult patients with SCEAD from 63 sites across 16 countries (2015-2022).
FMD was defined by history or imaging of cervical or renal arteries.
Logistic regression and Cox proportional hazards models were used to analyze correlates and recurrent SCEAD rates.

Main Results:

Of 3714 patients, 196 (5.3%) had FMD. FMD was associated with older age, female sex, noncervical dissection history, prior SCEAD, upper respiratory infection, cerebral aneurysm, and migraines.
Patients with FMD were less likely to have single vertebral artery dissection or occlusive dissection.
The 24-month risk of recurrent SCEAD was significantly higher in patients with FMD (7.7%) compared to those without (2.8%), with an adjusted hazard ratio of 2.75.

Conclusions:

FMD in patients with SCEAD is associated with specific clinical and radiological characteristics.
FMD significantly increases the risk of recurrent SCEAD, highlighting the importance of identifying FMD in these patients for risk assessment and management strategies.

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