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  • Title: [Fibromuscular dysplasia of the renal arteries].
    Author: Fiquet-Kempf B, Grimbert P, Pannier-Moreau I, Vuagnat A, Jeunemaitre X, Plouin PF.
    Journal: Nephrologie; 1999; 20(1):13-8. PubMed ID: 10081032.
    Abstract:
    Fibromuscular dysplasia is a non-atherosclerotic, non-inflammatory vascular disease that primary involves medium-sized and small arteries, most commonly the renal and carotid arteries. Dysplasic stenoses can be classified by angiography into three main subtypes, multifocal (multiple contiguous stenoses with the "string of beads" appearance), unifocal (single stenosis in a given renal artery), or tubular. The multifocal subtype is the most frequent and is usually associated with medial dysplasia, whereas unifocal and tubular stenoses are associated with intimal and perimedial dysplasia, respectively. Renovascular hypertension, mainly in women aged 30 to 50 years, is the most common manifestation of renal artery fibromuscular dysplasia. Its prevalence in hypertensive patients is estimated to less than 1 percent. The true prevalence of the disease is probably higher, however, because many cases can go undetected in normotensive or asymptomatic hypertensive patients. The first line treatment is percutaneous transluminal angioplasty that usually allows blood pressure improvement or normalization. Stenosis progression is slow and rarely leads to ischemic renal failure. Recognition of renal artery fibromuscular dysplasia should lead to screening for associated carotid artery lesions. Fibromuscular dysplasia can be a familial disease.
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