title: [Fibromuscular dysplasia].
abstract: Fibromuscular dysplasia is a segmentary, non-atherosclerotic, non-inflammatory vascular disease that may result in stenosis, occlusion, aneurysms or dissection of medium arteries. Renal involvement is the most frequent location, described in 60-100% of patients. Renal stenosis can be asymptomatic or complicated with arterial hypertension or less frequently with renal insufficiency. Carotid and vertebral involvements are less frequent (10-35%). Surgical management of fibromuscular dysplasia is now less common because of the better efficacy of percutaneous transluminal angioplasty. Thus, histologic characteristics are no longer relevant prognostic criteria. Clinical features and outcome vary according to angiographic presentation (focal or multifocal disease), with an increased recovery rate of hypertension with focal lesions. In the presence of renal fibromuscular dysplasia, only symptomatic patients are revascularized (recent or resistant hypertension) or patients with asymmetric renal size or impaired renal function. Transluminal angioplasty is the first-line treatment except for patients with complex lesions or stenosis associated with aneurysm.
authors.affiliation: Département de médecine interne et immunologie clinique, groupe hospitalier Pitié-Salpêtrière, AP-HP, 47-83, boulevard de l'Hôpital, 75013 Paris, France; Département hospitalo-universitaire I2B, université Pierre-et-Marie-Curie, Paris 6, 75013 Paris, France; CNRS, UMR 7211, 75005 Paris, France; Inserm, UMR S 959, 75013 Paris, France.
authors.forename: A C
authors.initials: AC
authors.lastname: Desbois
authors.affiliation: Service de chirurgie vasculaire, groupe hospitalier Pitié-Salpêtrière, AP-HP, 75013 Paris, France.
authors.forename: F
authors.initials: F
authors.lastname: Koskas
authors.affiliation: Département de médecine interne et immunologie clinique, groupe hospitalier Pitié-Salpêtrière, AP-HP, 47-83, boulevard de l'Hôpital, 75013 Paris, France; Département hospitalo-universitaire I2B, université Pierre-et-Marie-Curie, Paris 6, 75013 Paris, France; CNRS, UMR 7211, 75005 Paris, France; Inserm, UMR S 959, 75013 Paris, France. Electronic address: patrice.cacoub@psl.aphp.fr.
authors.forename: P
authors.initials: P
authors.lastname: Cacoub
mesh_terms: D000465:Algorithms; D005352:Fibromuscular Dysplasia / Q000175:diagnosis / Q000628:therapy; D006801:Humans
keywords: Aneurysm; Anévrysme; Artère rénale; Dysplasie fibromusculaire; Fibromuscular dysplasia; Renal artery; Stenosis; Sténose