Fiquet-Kempf B, Grimbert P, Pannier-Moreau I, Vuagnat A, Jeunemaitre X, Plouin P F
Service d'hypertension artérielle, Hôpital Broussais, Paris.
Nephrologie. 1999;20(1):13-8.
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.
纤维肌性发育异常是一种非动脉粥样硬化、非炎症性血管疾病，主要累及中、小动脉，最常见于肾动脉和颈动脉。发育异常性狭窄通过血管造影可分为三种主要亚型：多灶性（多个连续狭窄，呈“串珠样”外观）、单灶性（某一肾动脉单个狭窄）或管状。多灶性亚型最为常见，通常与中层发育异常有关，而单灶性和管状狭窄分别与内膜和中膜周围发育异常有关。肾血管性高血压主要见于30至50岁女性，是肾动脉纤维肌性发育异常最常见的表现。据估计，其在高血压患者中的患病率不到1%。然而，该病的实际患病率可能更高，因为许多病例在血压正常或无症状高血压患者中可能未被发现。一线治疗是经皮腔内血管成形术，通常可使血压改善或恢复正常。狭窄进展缓慢，很少导致缺血性肾衰竭。认识到肾动脉纤维肌性发育异常应促使对相关颈动脉病变进行筛查。纤维肌性发育异常可能是一种家族性疾病。
