Pamela Mace

Clinical Manifestations of Fibromuscular Dysplasia Vary by Patient Sex: A Report of the United States Registry for Fibromuscular Dysplasia

To the Editor: Fibromuscular dysplasia (FMD) is an uncommon arteriopathy which can result in stenosis, aneurysm, dissection, and/or occlusion of arteries. It most commonly affects the renal, extracranial carotid, and vertebral arteries but can affect any artery. Although FMD occurs primarily in

Smoking and Adverse Outcomes in Fibromuscular Dysplasia: U.S. Registry Report.

The pathophysiology of fibromuscular dysplasia (FMD) is unknown; however, smoking has been implicated as a potential contributing factor [(1,2)][1]. Prior studies have shown a higher prevalence of smoking among those with renal FMD compared with matched hypertensive control subjects [(3)][2]. The

Prevalence of Intracranial Aneurysm in Women With Fibromuscular Dysplasia: A Report From the US Registry for Fibromuscular Dysplasia

Importance The prevalence of intracranial aneurysm in patients with fibromuscular dysplasia (FMD) is uncertain. Objective To examine the prevalence of intracranial aneurysm in women diagnosed with FMD. Design, Setting, and Participants This cross-sectional study included 669 women with intracranial imaging registered in the US Registry for Fibromuscular Dysplasia, an observational disease-based registry of patients with FMD confirmed by vascular imaging and currently enrolling at 14 participating US academic centers. Registry enrollment began in 2008, and data were abstracted in September 2015. Patients younger than 18 years at the time of FMD diagnosis were excluded. Imaging reports of all patients with reported internal carotid, vertebral, or suspected intracranial artery aneurysms were reviewed. Only saccular or broad-based aneurysms 2 mm or larger in greatest dimension were included. Extradural aneurysms in the internal carotid artery were included; fusiform aneurysms, infundibulae, and vascular segments with uncertainty were excluded. Main Outcomes and Measures Percentage of women with FMD with intracranial imaging who had an intracranial aneurysm. Results Of 1112 female patients in the registry, 669 (60.2%) had undergone intracranial imaging at the time of enrollment (mean [SD] age at enrollment, 55.6 [10.9] years). Of the 669 patients included in the analysis, 86 (12.9%; 95% CI, 10.3%-15.9%) had at least 1 intracranial aneurysm. Of these 86 patients, 25 (53.8%) had more than 1 intracranial aneurysm. Intracranial aneurysms 5 mm or larger occurred in 32 of 74 patients (43.2%), and 24 of 128 intracranial aneurysms (18.8%) were in the posterior communicating or posterior arteries. The presence of intracranial aneurysm did not vary with location of extracranial FMD involvement. A history of smoking was significantly associated with intracranial aneurysm: 42 of 78 patients with intracranial aneurysm (53.8%) had a smoking history vs 163 of 564 patients without intracranial aneurysm (28.9%; P < .001). Conclusions and Relevance The prevalence of intracranial aneurysm in women diagnosed with FMD is significantly higher than reported in the general population. Although the clinical benefit of screening for intracranial aneurysm in patients with FMD has yet to be proven, these data lend support to the recommendation that all patients with FMD undergo intracranial imaging if not already performed.

ANEURYSM AND DISSECTION IN FIBROMUSCULAR DYSPLASIA: FINDINGS FROM THE UNITED STATES REGISTRY FOR FMD

The clinical phenotype and prognosis in Fibromuscular Dysplasia (FMD) patients (pts) with Dissection (D) and Aneurysm (A) are poorly defined. Clinical features, locations and therapeutic procedures were reviewed in 732 pts enrolled in the United States Registry for FMD. Aneurysm (A) was present in

CLINICAL FEATURES AND PRESENTING SYMPTOMS AND SIGNS OF FIBROMUSCULAR DYSPLASIA: A REPORT OF THE FIBROMUSCULAR DYSPLASIA PATIENT REGISTRY

Results: Most pts were female (91.4%) with a mean age at diagnosis of 51.5 + 14.3 years. The most common presenting symptoms and signs are shown below; only 4.3% were asymptomatic. The median interval from first symptom to diagnosis was within the first year (IQR 0-4 years). Family history (FH) of stroke (51.7%) or aneurysm (22.7%) was common, but FH of diagnosed FMD was uncommon (7.9%). Ultrasound was the most common diagnostic modality, followed by angiography, CTA, and MRA. The most common vascular beds involved were: renal (61.3%), extracranial carotid (55.3%), vertebral (16.6%), mesenteric (11.3%), lower extremity (8.0%), intracranial (7.3%). Medial fibroplasia was the most common type, followed by intimal disease.

PERIPHERAL ARTERY DISSECTION IN PATIENTS WITH FIBROMUSCULAR DYSPLASIA: A REPORT FROM THE UNITED STATES FIBROMUSCULAR DYSPLASIA PATIENT REGISTRY

Results: Of the 447 patients entered into the FMD registry, 88 (19.7%) had a dissection of a peripheral artery [carotid (68%), renal (22%), vertebral (18%), mesenteric (7%) and other arteries (6.1%)]. Twenty percent of patients had more than one dissection with a maximum of 3. The differences between those patients with and without dissection are shown in the Table. Dissection