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Association of multifocal fibromuscular dysplasia in elderly patients with a more benign clinical phenotype: Data from the US Registry for Fibromuscular Dysplasia

JAMA Cardiology Aug 22, 2018

Bagh I, et al. - In this multicenter registry study, researchers focused on the variance in clinical presentation, arterial bed involvement, vascular events, and requirement for vascular procedures between younger and older patients with fibromuscular dysplasia (FMD) in the US Registry for FMD. They noted that, compared with younger patients, patients 65 years or older at the time of diagnosis of multifocal FMD were more likely to be asymptomatic, had lower prevalence of major vascular events, and had undergone fewer therapeutic vascular procedures. A more benign phenotype and fewer symptoms could be seen in patients with multifocal FMD diagnosed at an older age.

Methods

  • Researchers performed an analysis of baseline data for patients enrolled in the US Registry for FMD as of December 15, 2016, at referral centers participating in the US Registry for FMD.
  • Participants were patients 18 years and older at the time of enrollment and those with only confirmed multifocal (string of beads type) FMD.
  • Categories of patients were formed according to age at the time of diagnosis (≥65 years vs <65 years).
  • Main outcomes and measures included prevalence of specific symptoms, vascular events, and prior vascular procedures at the time of enrollment in the registry.

Results

  • Of 1,016 patients included, 170 (16.7%) were 65 years or older at the time of diagnosis.
  • The likeliness to be asymptomatic at the time of diagnosis (4.2% vs 1.4%; P=.02) was more in older patients with FMD.
  • Older patients less commonly had headache and pulsatile tinnitus, both of which were common manifestations of FMD (40.5% vs 69.1%; P<.001 and 30% vs 44.6%; P<.001, respectively).
  • More commonly, involvement of extracranial carotid arteries was observed in patients 65 years or older at time of diagnosis (87% vs 79.4%; P=.03).
  • Findings revealed no difference in prevalence of renal artery involvement, number of arterial beds involved, or diagnosis of any aneurysm.
  • They also found that patients 65 years or older were less likely to have had a major vascular event (37.1% vs 46.1%; P=.03) and fewer had undergone a therapeutic vascular procedure (18.5% vs 33.1%; P<.001).

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