The United States registry for fibromuscular dysplasia: New findings and breaking myths

摘要

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.