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Clinical manifestations and diagnosis of fibromuscular dysplasia

Author
Jeffrey W Olin, DO
Section Editor
George L Bakris, MD
Deputy Editor
John P Forman, MD, MSc

INTRODUCTION

Fibromuscular dysplasia (FMD) is a noninflammatory, nonatherosclerotic disorder that leads to arterial stenosis, occlusion, aneurysm, dissection, and arterial tortuosity. These findings have been observed in nearly every arterial bed. The most frequently involved arteries are the renal and internal carotid arteries, followed by the vertebral, visceral, and external iliac arteries [1]. Disease presentation may vary widely, depending upon the arterial segment involved and the severity of disease.

The epidemiology, pathogenesis, clinical manifestations, and diagnosis of FMD in adults are reviewed here. The treatment of FMD and the general evaluation for possible renovascular hypertension and stroke are discussed separately. (See "Treatment of fibromuscular dysplasia of the renal arteries" and "Establishing the diagnosis of renovascular hypertension" and "Evaluation of secondary hypertension" and "Clinical diagnosis of stroke subtypes" and "Overview of the evaluation of stroke".)

EPIDEMIOLOGY

Patients with FMD have involvement of the renal arteries approximately 75 to 80 percent of the time and involvement of the extracranial cerebrovascular arteries (eg, carotid and vertebral arteries) approximately 75 percent of the time [1]. Approximately two-thirds of patients have multiple arteries involved [2-4].

Among adults, FMD is more common among females. In most large series, approximately 90 percent of cases are in women. There does not appear to be a female predominance among children with FMD [5].

In the past, it was believed that FMD was a disease of young women. However, older individuals account for a large proportion of affected patients in several cohorts. As an example, in the United States FMD Registry, the mean age at diagnosis was 52 years, with a range of 5 to 86 years [1,6,7].

                     
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Literature review current through: Nov 2017. | This topic last updated: Nov 30, 2017.
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