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Fibromuscular dysplasia: its various phenotypes in everyday practice in 2021

Patricia Van der Niepen1, Tom Robberechts2, Hannes Devos3

  • 1Department of Nephrology and Hypertension, Universitair Ziekenhuis Brussel, Vrije Universiteit Brussel, Brussels, Belgium. patricia.vanderniepen@uzbrussel.be.

Kardiologia Polska
|June 24, 2021
PubMed

Insights

Fibromuscular dysplasia (FMD) is a vascular disease affecting arteries. This review focuses on the clinical management of adult FMD, covering its manifestations, diagnosis, and treatment strategies.

Area of Science:

  • Vascular Medicine
  • Cardiology
  • Genetics

Background:

  • Fibromuscular dysplasia (FMD) is a non-atherosclerotic vascular disease affecting medium-sized arteries.
  • Its pathogenesis is not fully understood, likely involving genetic and environmental factors.
  • FMD commonly affects women, but men may experience more severe disease, particularly if they smoke.

Purpose of the Study:

  • To review the clinical management of adult Fibromuscular dysplasia (FMD) in daily practice.
  • To provide an in-depth understanding of FMD's clinical manifestations, epidemiology, natural history, and pathogenesis.
  • To guide clinicians in diagnosing and managing FMD based on vascular phenotype and clinical presentation.

Main Methods:

  • Review of current literature on Fibromuscular dysplasia (FMD).
  • Analysis of diagnostic approaches including CT-, MR-, and catheter-based angiography.
  • Synthesis of management strategies tailored to FMD phenotypes and clinical scenarios.

Main Results:

  • FMD presents with diverse phenotypes, including 'string of beads,' stenosis, aneurysms, dissections, and tortuosity.
  • Common clinical presentations include arterial hypertension and neurological symptoms due to renal and cerebrovascular involvement.
  • Diagnosis relies on angiography, though silent lesions can be incidentally found.

Conclusions:

  • Effective management of FMD requires consideration of the specific vascular phenotype and clinical presentation.
  • Continued research is crucial for advancing the understanding and treatment of FMD.
  • This review offers practical insights for the daily clinical management of adult FMD.
Keywords:
carotid artery dissectionfibromuscular dysplasiarenal artery stenosisspontaneous coronary artery dissectionvisceral FMD

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