Expanding Clinical Phenotype of Fibromuscular Dysplasia
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See related article, pp 652–658
Before 2004, fibromuscular dysplasia (FMD) was thought to predominately affect the renal arteries.1 In the early studies, the extracranial carotid and vertebral arteries were involved in only 25% to 30% of patients with FMD.1 However, in a recent report, renal artery involvement occurred in 75.3% of 507 patients, extracranial carotid artery in 73.7% of 476 patients, and vertebral artery in 33.4% of 329 patients.2 There are now >1600 patients enrolled in the US registry, and it seems that the phenotypic presentation is expanding to include not only stenosis (multifocal [string of beads] or focal [a discrete area of a single stenosis]) but also aneurysm, dissection, and arterial tortuosity. Although other studies have reported on the frequency of multivessel FMD,3,4 Plouin et al5 have conducted the first prospective study in which cross-sectional imaging (computed tomographic angiogram [CTA] or magnetic resonance angiogram [MRA]) was performed in every patient enrolled in the ARCADIA FMD registry (Assessment of Renal and Cervical Artery Dysplasia). Patients with renal artery FMD underwent CTA or MRA from the aortic arch to the intracranial vessels, and patients with extracranial cerebrovascular FMD underwent CTA or MRA from the diaphragm to the pelvis. In the ARCADIA FMD registry, at least 2 vascular beds were affected by dysplastic stenosis in 48% of participants. However, if a patient had stenosis in 1 vascular bed and either aneurysm or dissection in another vascular bed, they were coded as having single vascular …