Contemporary management of fibromuscular dysplasia

Fibromuscular dysplasia is an underdiagnosed and misunderstood disease. The purpose of this review is to inform healthcare providers and the public about a condition that may be more common than previously thought.

There has been little new information published about fibromuscular dysplasia in the past 30 years. The International Registry that is now underway will remedy that situation and provide a large number of patients to study with this condition.

Fibromuscular dysplasia is a noninflammatory, nonatherosclerotic disease that has been reported in almost every arterial bed and primarily affects women aged 15-50 years. It most commonly presents in the renal and extracranial cerebrovascular arteries, either manifesting as hypertension, transient ischemic attack or stroke, respectively. Some patients may be asymptomatic and fibromuscular dysplasia could only be discovered by imaging for some other reason or by the detection of an asymptomatic bruit. Dissection or aneurysm may also occur in patients with fibromuscular dysplasia. The true prevalence is unknown, partially because of the fact that it is underdiagnosed in many patients. Treatment consists of antiplatelet therapy for asymptomatic individuals and percutaneous balloon angioplasty for patients with indications for intervention. Patients with macroaneurysms should be treated with either a covered stent or surgery.