Fibromuscular dysplasia; arterial dissections; spontaneous coronary artery dissection; Internal Medicine
Abstract :
[en] Fibromuscular dysplasia (FMD) is an idiopathic and systemic non-inflammatory and non-atherosclerotic arterial disease. Fifteen to 25 % of patients with FMD present with arterial dissection in at least one arterial bed. Conversely, a substantial number of patients with renal, carotid and visceral dissection have underlying FMD. Also, while few patients with FMD develop coronary artery dissection, lesions suggestive of multifocal FMD have been reported in 30 to 80% of patients with Spontaneous Coronary Artery Dissection (SCAD), and the relation between these two entities remains controversial. The frequent association of FMD with arterial dissection, both in coronary and extra-coronary arteries raises a number of practical and theoretical questions: (i) are FMD and arterial dissections two different facets of the same disease or distinct though related entities? (ii) is SCAD just a manifestation of coronary FMD or a different disease? (iii) What is the risk and which are predictive factors of developing arterial dissection in a patient with FMD? (iv) What proportion of patients who experienced an arterial dissection have underlying FMD, and does this finding influence the risk of subsequent arterial complications? In this review we will address these different questions using fragmentary, mostly cross-sectional evidence derived from large registries and studies from Europe and the United States, as well as arguments derived from demographics, clinical presentation, imaging, and when available histology and genetics. From there we will derive practical consequences for nosology, screening and follow-up.
Disciplines :
Urology & nephrology
Author, co-author :
Huart, Justine ; Centre Hospitalier Universitaire de Liège - CHU > > Service de néphrologie
Stoenoiu, Maria S; Institut de Recherche Expérimentale et Clinique, Université catholique de Louvain, Brussels, Belgium
Zedde, Marialuisa; Neurology Unit, Stroke Unit, AUSL-IRCCS di Reggio Emilia, Reggio Emilia, Italy
Pascarella, Rosario; Neuroradiology Unit, AUSL-IRCCS di Reggio Emilia, Reggio Emilia, Italy
Adlam, David; Department of Cardiovascular Sciences, University of Leicester and NIHR Leicester Biomedical Research Centre, Leicester, United Kingdom
Persu, Alexandre ; Division of Cardiology, Cliniques Universitaires Saint-Luc, Université catholique de Louvain, Brussels, Belgium ; Pole of Cardiovascular Research, Institut de Recherche Expérimentale et Clinique, Université catholique de Louvain, Brussels, Belgium
Language :
English
Title :
From Fibromuscular Dysplasia to Arterial Dissection And Back.
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