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Article

Fibromuscular dysplasia: Advances in understanding and management

Ellen K. Brinza, MS and Heather L. Gornik, MD
Cleveland Clinic Journal of Medicine November 2016, 83 (11 suppl 2) S45-S51; DOI: https://doi.org/10.3949/ccjm.83.s2.06
Ellen K. Brinza
Vascular Medicine Section, Department of Cardiovascular Medicine, Cleveland Clinic
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Heather L. Gornik
Vascular Medicine Section, Department of Cardiovascular Medicine, Cleveland Clinic
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  • FIGURE 1
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    FIGURE 1

    Multifocal fibromuscular dysplasia (FMD) involving the internal carotid artery (A) and a renal artery (B) with a “string-of-beads” appearance. The less common type, focal FMD, involving the internal carotid artery (C) and a renal artery (D).

    Reprinted with permission from Wolters Kluwer Health, Inc. (Poloskey SL, et al. Fibromuscular dysplasia. Circulation 2012; 125:e636–e639).

  • FIGURE 2
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    FIGURE 2

    (A) Duplex ultrasonography with color power angiography in a patient with fibromuscular dysplasia shows arterial tortuosity (the “S curve”) in the internal carotid artery and areas of beading. This feature can also be seen in renal arteries, as shown on angiography (B).

  • FIGURE 3
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    FIGURE 3

    Spontaneous coronary artery dissection (SCAD) in a 42-year-old woman with fibromuscular dysplasia (FMD) who presented with chest pain and nausea and non-ST- segment elevation myocardial infarction. She was found with coronary angiography to have SCAD of the left circumflex coronary (A, red arrow). Computed tomographic angiography showed a string-of-beads appearance of the left internal carotid artery (B, red arrow). Duplex ultrasonography showed turbulence and tortuosity in the mid to distal left internal carotid artery, consistent with a diagnosis of multifocal carotid FMD (C).

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    TABLE 1

    Key findings of publications from the US Registry for Fibromuscular Dysplasia

    Olin et al8 (2012)
    • First publication from the US Registry

    • Extracranial carotid FMD is as common as renal FMD

    • Defined common symptoms, including hypertension, headache, and pulsatile tinnitus

    Kim et al10 (2013)
    • Only 8.5% of registrants were male

    • Renal involvement was higher in men than in women (89.7% vs 74.1%); extracranial involvement was more common in women than in men (74.9% vs 44.1%)

    • Compared with women, men were twice as likely to have an aneurysm (40.8% vs. 20.4%) or dissection (39.6% vs 20.0%)

    • Renal artery dissection with flank pain and infarction was a pattern of disease presentation in men with FMD

    Weinberg et al11 (2015)
    • High rate of antiplatelet and antihypertensive therapy among registrants

    • 72.9% of FMD patients were on antiplatelet therapy (58.8% on aspirin alone, 4.6% on clopidogrel only)

    • 71.7% of patients were on antihypertensive medications, with 21.5% on three or more

    Green et al12 (2016)
    • Mean age at the time of diagnosis in pediatric patients was 8.4 ± 4.8 years

    • Significantly more male patients in the pediatric FMD population vs the adult FMD population (42.2% vs 6.0%)

    • Compared with adults, pediatric patients more likely to have renal artery involvement (97% vs 69.7%) and mesenteric artery involvement (38.9% vs 16.2%)

    • More pediatric FMD patients than adult FMD patients reported having a family member with FMD (17.2% vs 4.7%)

    Kadian-Dodov et al7 (2016)
    • 21.7% of patients in the registry had an aneurysm, 25.7% had a dissection, and 41.7% had an aneurysm or dissection

    • Roughly one-third of patients with aneurysm required intervention, most commonly for those found in the extracranial carotid, renal, and intracranial arteries

    O’Connor et al3 (2016)
    • 34.5% of patients in the registry were current or former smokers

    • Smoking history was associated with worse outcomes than in nonsmokers, including an increased need for revascularization (45.9% vs 36.7%), and a higher likelihood of aneurysm (24.8% vs 18.9%) and adverse symptoms such as claudication (15.1% vs 7.4%)

    • FMD = fibromuscular dysplasia

    • Data from references 3, 7, 8, 10–12.

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Cleveland Clinic Journal of Medicine: 83 (11 suppl 2)
Cleveland Clinic Journal of Medicine
Vol. 83, Issue 11 suppl 2
1 Nov 2016
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Fibromuscular dysplasia: Advances in understanding and management
Ellen K. Brinza, Heather L. Gornik
Cleveland Clinic Journal of Medicine Nov 2016, 83 (11 suppl 2) S45-S51; DOI: 10.3949/ccjm.83.s2.06

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Fibromuscular dysplasia: Advances in understanding and management
Ellen K. Brinza, Heather L. Gornik
Cleveland Clinic Journal of Medicine Nov 2016, 83 (11 suppl 2) S45-S51; DOI: 10.3949/ccjm.83.s2.06
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    • ABSTRACT
    • DISTINCT FROM ATHEROSCLEROSIS
    • THE US REGISTRY FOR FMD
    • EPIDEMIOLOGY AND PATHOPHYSIOLOGY
    • A BROADER SCOPE OF ARTERIAL MANIFESTATIONS
    • APPROACH TO MANAGEMENT
    • CONCLUSION
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