The United States Registry for Fibromuscular Dysplasia: Results in the First 447 Patients
Background—Fibromuscular dysplasia (FMD), a non-inflammatory disease of medium sized arteries, may lead to stenosis, occlusion, dissection and/or aneurysm. There has been little progress in understanding the epidemiology, pathogenesis and outcomes since its first description in 1938.
Methods and Results—Clinical features, presenting symptoms, and vascular events are reviewed for the first 447 patients enrolled in a national FMD registry from 9 U.S. sites. Vascular beds were imaged selectively based on clinical presentation and local practice. The majority of patients were female (91%) with a mean age at diagnosis of 51.9 (SD 13.4 years, range 5-83 years). Hypertension, headache, and pulsatile tinnitus were the most common presenting symptoms of the disease. Self-reported family history of stroke (53.5%), aneurysm (23.5%) and sudden death (19.8%) were common, but FMD in first or second degree relatives was reported only in 7.3%. FMD was identified in the renal artery in 294 patients, extracranial carotid arteries in 251 patients, vertebral arteries in 82 patients. A past or presenting history of vascular events were common: 19.2% of patients had a transient ischemic attack or stroke, 19.7% had suffered arterial dissection(s), and 17% of patients had an aneurysm(s). The most frequent indications for therapy were hypertension, aneurysm, and dissection.
Conclusions—In this registry, FMD occurred primarily in middle-aged women, though it presents across the lifespan. Cerebrovascular FMD occurred as frequently as renal FMD. While a significant proportion of FMD patients may present with a serious vascular event, many present with nonspecific symptoms and a subsequent delay in diagnosis.
- Received January 9, 2012.
- Accepted May 4, 2012.
- Copyright © 2012, American Heart Association, Inc. All rights reserved. Unauthorized use prohibited