Current progress in clinical, molecular, and genetic aspects of adult fibromuscular dysplasia.
Animals
Arteries
/ metabolism
Biomedical Research
/ trends
Fibromuscular Dysplasia
/ diagnosis
Gene Expression Profiling
/ trends
Genetic Predisposition to Disease
Hemodynamics
Humans
Molecular Diagnostic Techniques
/ trends
Phenotype
Predictive Value of Tests
Prognosis
Proteomics
/ trends
Risk Assessment
Risk Factors
Vascular Remodeling
Fibromuscular dysplasia
Genomic
Proteomic
Research
Spontaneous dissection
Journal
Cardiovascular research
ISSN: 1755-3245
Titre abrégé: Cardiovasc Res
Pays: England
ID NLM: 0077427
Informations de publication
Date de publication:
07 01 2022
07 01 2022
Historique:
received:
18
10
2020
accepted:
17
03
2021
pubmed:
20
3
2021
medline:
8
3
2022
entrez:
19
3
2021
Statut:
ppublish
Résumé
Fibromuscular dysplasia (FMD) is a non-atherosclerotic vascular disease that may involve medium-sized muscular arteries throughout the body. The majority of FMD patients are women. Although a variety of genetic, mechanical, and hormonal factors play a role in the pathogenesis of FMD, overall, its cause remains poorly understood. It is probable that the pathogenesis of FMD is linked to a combination of genetic and environmental factors. Extensive studies have correlated the arterial lesions of FMD to histopathological findings of arterial fibrosis, cellular hyperplasia, and distortion of the abnormal architecture of the arterial wall. More recently, the vascular phenotype of lesions associated with FMD has been expanded to include arterial aneurysms, dissections, and tortuosity. However, in the absence of a string-of-beads or focal stenosis, these lesions do not suffice to establish the diagnosis. While FMD most commonly involves renal and cerebrovascular arteries, involvement of most arteries throughout the body has been reported. Increasing evidence highlights that FMD is a systemic arterial disease and that subclinical alterations can be found in non-affected arterial segments. Recent significant progress in FMD-related research has led to improve our understanding of the disease's clinical manifestations, natural history, epidemiology, and genetics. Ongoing work continues to focus on FMD genetics and proteomics, physiological effects of FMD on cardiovascular structure and function, and novel imaging modalities and blood-based biomarkers that can be used to identify subclinical FMD. It is also hoped that the next decade will bring the development of multi-centred and potentially international clinical trials to provide comparative effectiveness data to inform the optimal management of patients with FMD.
Identifiants
pubmed: 33739371
pii: 6178787
doi: 10.1093/cvr/cvab086
pmc: PMC8752362
doi:
Types de publication
Journal Article
Research Support, N.I.H., Extramural
Research Support, Non-U.S. Gov't
Review
Langues
eng
Sous-ensembles de citation
IM
Pagination
65-83Subventions
Organisme : NHLBI NIH HHS
ID : R01 HL139672
Pays : United States
Organisme : NHLBI NIH HHS
ID : R01 HL122684
Pays : United States
Organisme : NHLBI NIH HHS
ID : R01 HL148167
Pays : United States
Organisme : NHLBI NIH HHS
ID : R01 HL086694
Pays : United States
Organisme : Department of Health
Pays : United Kingdom
Informations de copyright
Published on behalf of the European Society of Cardiology. All rights reserved. © The Author(s) 2021. For permissions, please email: journals.permissions@oup.com.
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