open access

Online first
Review paper
Published online: 2021-06-22
Get Citation

Fibromuscular dysplasia: its various phenotypes in everyday practice in 2021

Patricia Van der Niepen, Tom Robberechts, Hannes Devos, Frank van Tussenbroek, Andrzej Januszewicz, Alexandre Persu
DOI: 10.33963/KP.a2021.0040
·
Pubmed: 34166522

open access

Online first
Review
Published online: 2021-06-22

Abstract

Fibromuscular dysplasia (FMD) is a non-atherosclerotic vascular disease that may involve medium-sized muscular arteries throughout the body. The pathogenesis of FMD remains poorly understood, but a combination of genetic and environmental factors may be involved. The majority of FMD patients are women, but men may have a more progressive disease, especially when smoking. Besides the classical phenotype of string of beads or focal stenosis, arterial aneurysms, dissections, and tortuosity are frequent manifestations of the disease. However, the differential diagnosis of FMD is extensive and includes imaging artefacts as well as other arterial diseases. Diagnosis is based on CT-, MR-, or conventional catheter-based angiography during work-up of clinical manifestations, but clinically silent lesions may be found incidentally. Arterial hypertension and neurological symptoms are the most frequent clinical presentations, as renal and cerebrovascular arteries are the most commonly involved. However, involvement of most arteries throughout the body has been reported, resulting in a variety of clinical symptoms. The management of FMD depends on the vascular phenotype as well on the clinical picture. Ongoing FMD-related research will elaborate in depth the current progress in improved understandings of the disease's clinical manifestations, epidemiology, natural history and pathogenesis. This review is focused on the clinical management of adult FMD in daily practice.

Abstract

Fibromuscular dysplasia (FMD) is a non-atherosclerotic vascular disease that may involve medium-sized muscular arteries throughout the body. The pathogenesis of FMD remains poorly understood, but a combination of genetic and environmental factors may be involved. The majority of FMD patients are women, but men may have a more progressive disease, especially when smoking. Besides the classical phenotype of string of beads or focal stenosis, arterial aneurysms, dissections, and tortuosity are frequent manifestations of the disease. However, the differential diagnosis of FMD is extensive and includes imaging artefacts as well as other arterial diseases. Diagnosis is based on CT-, MR-, or conventional catheter-based angiography during work-up of clinical manifestations, but clinically silent lesions may be found incidentally. Arterial hypertension and neurological symptoms are the most frequent clinical presentations, as renal and cerebrovascular arteries are the most commonly involved. However, involvement of most arteries throughout the body has been reported, resulting in a variety of clinical symptoms. The management of FMD depends on the vascular phenotype as well on the clinical picture. Ongoing FMD-related research will elaborate in depth the current progress in improved understandings of the disease's clinical manifestations, epidemiology, natural history and pathogenesis. This review is focused on the clinical management of adult FMD in daily practice.

Get Citation

Keywords

fibromuscular dysplasia, carotid artery dissection, renal artery stenosis, spontaneous coronary artery dissection, visceral FMD

About this article
Title

Fibromuscular dysplasia: its various phenotypes in everyday practice in 2021

Journal

Kardiologia Polska (Polish Heart Journal)

Issue

Online first

Article type

Review paper

Published online

2021-06-22

DOI

10.33963/KP.a2021.0040

Pubmed

34166522

Keywords

fibromuscular dysplasia
carotid artery dissection
renal artery stenosis
spontaneous coronary artery dissection
visceral FMD

Authors

Patricia Van der Niepen
Tom Robberechts
Hannes Devos
Frank van Tussenbroek
Andrzej Januszewicz
Alexandre Persu

Important: This website uses cookies. More >>

The cookies allow us to identify your computer and find out details about your last visit. They remembering whether you've visited the site before, so that you remain logged in - or to help us work out how many new website visitors we get each month. Most internet browsers accept cookies automatically, but you can change the settings of your browser to erase cookies or prevent automatic acceptance if you prefer.

By VM Media Sp. z o.o. VM Group Sp.k., ul. Świętokrzyska 73 , 80–180 Gdańsk, Poland

phone:+48 58 320 94 94, fax:+48 58 320 94 60, e-mail: viamedica@viamedica.pl