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The United States Registry for Fibromuscular Dysplasia: Results in the First 447 Patients
Study Questions:
What are the epidemiology, clinical characteristics, management, and outcomes of patients with fibromuscular dysplasia (FMD)?
Methods:
Clinical features, presenting symptoms, and vascular events are reviewed for the first 447 patients enrolled in a national FMD registry from nine US sites. Vascular beds were imaged selectively based on clinical presentation and local practice. A standardized FMD data form of definitions was used for each component of the data collection form.
Results:
The majority of patients were female (91%), with a mean age at diagnosis of 51.9 (standard deviation, 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, and vertebral arteries in 82 patients. A past or presenting history of vascular events was common: 19.2% of patients had a transient ischemic attack or stroke, 19.7% had experienced arterial dissection(s), and 17% of patients had an aneurysm(s). The most frequent indications for therapy were hypertension, aneurysm, and dissection.
Conclusions:
The authors concluded that although a significant proportion of FMD patients may present with a serious vascular event, many present with nonspecific symptoms and a subsequent delay in diagnosis.
Perspective:
This multicenter registry provides data on the largest cohort of FMD patients to date. Overall, FMD is a nonatherosclerotic, noninflammatory vascular disease that primarily affects women in the prime of their life. It most commonly affects the renal, carotid, and vertebral arteries, but may occur in virtually any artery of the body. Multivessel involvement is also common. The most common clinical manifestations of FMD are hypertension, headaches, pulsatile tinnitus, and dizziness, but dissection, aneurysm, transient ischemic attack, and stroke also occur with a high frequency. A cerebrovascular event including transient ischemic attack, stroke, and/or amaurosis fugax occur in one of every four patients with FMD. The presence of a carotid bruit in a patient under age 60 or an epigastric bruit in a patient with hypertension should alert the clinician to the possible diagnosis of FMD. Earlier diagnosis may prevent the consequences of poorly controlled hypertension, and allow for the identification of aneurysms and dissections and optimize treatment.
Keywords: Amaurosis Fugax, Stroke, Ischemic Attack, Transient, Tinnitus, Vascular Diseases, Death, Sudden, Carotid Arteries, Vertigo, Dizziness, Fibromuscular Dysplasia, United States, Hypertension
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