Plouin Pierre-François, Fiquet Béatrice, Bobrie Guillaume, Jeunemaître Xavier
Unité d'hypertension artérielle, centre de référence des maladies vasculaires rares, hôpital européen Georges-Pompidou, 20, rue Leblanc, 75015 Paris, France.
Unité d'hypertension artérielle, centre de référence des maladies vasculaires rares, hôpital européen Georges-Pompidou, 20, rue Leblanc, 75015 Paris, France.
Nephrol Ther. 2016 Apr;12 Suppl 1:S135-8. doi: 10.1016/j.nephro.2016.02.011. Epub 2016 Mar 8.
Fibromuscular dysplasia is non-atherosclerotic, non-inflammatory disease of the medium caliber arteries causing segmental stenosis, and sometimes aneurysm and/or dissection. Renal involvement is either asymptomatic or revealed by hypertension, rarely acute complications (renal infarction/hemorrhage). Cross-sectional imaging or angiography differentiates multifocal fibromuscular dysplasia (pearl necklace appearance) and focal fibromuscular dysplasia (tubular stenosis). Several differential diagnoses are to be mentioned. Carotid and vertebral involvement are possible. Smoking cessation must be encouraged. Selected patients benefit from renal revascularization. The best indications are recent or resistant hypertension, and progressive renal atrophy. Angioplasty without stent revascularization is the technique of choice in purely stenotic forms.
纤维肌发育不良是一种发生于中等管径动脉的非动脉粥样硬化、非炎症性疾病，可导致节段性狭窄，有时还会出现动脉瘤和/或夹层。肾脏受累时可无症状，或表现为高血压，急性并发症（肾梗死/出血）罕见。横断面成像或血管造影可区分多灶性纤维肌发育不良（珍珠项链样表现）和局灶性纤维肌发育不良（管状狭窄）。需要提及几种鉴别诊断。颈动脉和椎动脉也可能受累。必须鼓励戒烟。部分患者可从肾血运重建中获益。最佳适应证是近期发生的或难治性高血压以及进行性肾萎缩。对于单纯狭窄型，无支架血管成形术是首选技术。
