Sharma Aditya M, Kline Bryan
Division of Cardiovascular Medicine (Vascular Medicine), Department of Medicine University of Virginia School of Medicine, Charlottesville, VA.
Medical Education, University of Virginia School of Medicine, Charlottesville, VA.
Tech Vasc Interv Radiol. 2014 Dec;17(4):258-63. doi: 10.1053/j.tvir.2014.11.007. Epub 2014 Nov 13.
Fibromuscular dysplasia (FMD) is a nonatherosclerotic, noninflammatory arterial disease predominantly seen in women. Most FMD cases are classified as medial fibroplasia, which has the appearance of a string of beads on angiography. Until recently, FMD was thought to be seen in women in their 20s and 30s, with more than 60%-75% of cases occurring in the renal artery and 25% of cases in the carotid artery. Hypertension was regarded as the primary symptom seen in these patients followed by a small number of patients presenting with intracranial aneurysms with subarachnoid hemorrhage. The United States Registry of FMD, a patient registry, has broken many preconceived notions as well as provided more in-depth knowledge of this uncommon disorder. In this review, we discuss the findings of this registry and its use in understanding this disorder. In addition to the registry, we review other recent studies and future directions in the diagnosis and management of this disorder.
纤维肌发育不良（FMD）是一种主要见于女性的非动脉粥样硬化、非炎症性动脉疾病。大多数FMD病例被归类为中膜纤维增生，在血管造影上表现为一串珠子。直到最近，FMD还被认为多见于二三十岁的女性，超过60%-75%的病例发生在肾动脉，25%的病例发生在颈动脉。高血压被视为这些患者的主要症状，其次是少数出现蛛网膜下腔出血的颅内动脉瘤患者。美国FMD患者登记处打破了许多先入之见，也提供了对这种罕见疾病更深入的认识。在这篇综述中，我们讨论了该登记处的研究结果及其在理解这种疾病中的应用。除了该登记处，我们还综述了其他近期研究以及这种疾病诊断和管理的未来方向。
