Diagnosis, management, and future developments of fibromuscular dysplasia

Abstract

Fibromuscular dysplasia (FMD) is a nonatherosclerotic noninflammatory vascular disease that primarily affects women from age 20 to 60, but may also occur in infants and children, men, and the elderly. It most commonly affects the renal and carotid arteries but has been observed in almost every artery in the body. FMD has been considered rare and thus is often underdiagnosed and poorly understood by many health care providers. There are, however, data to suggest that FMD is much more common than previously thought, perhaps affecting as many as 4% of adult women. When it affects the renal arteries, the most common presentation is hypertension. When it affects the carotid or vertebral arteries, the patient may present with transient ischemic attack or stroke, or dissection. An increasing number of patients are asymptomatic and are only discovered incidentally when imaging is performed for some other reason or by the detection of an asymptomatic bruit. FMD should be considered in the differential diagnosis of a young person with a cervical bruit; a "swishing" sound in the ear(s); transient ischemic attack, stroke, or dissection of an artery; or in individuals aged ≤ 35 years with onset hypertension. Treatment consists of antiplatelet therapy for asymptomatic individuals and percutaneous balloon angioplasty for patients with indications for intervention. Patients with aneurysms should be treated with a covered stent or open surgical repair. Little new information has been published about FMD in the last 40 years. The recently instituted International Registry for Fibromuscular Dysplasia will remedy that situation and provide observational data on a large numbers of patients with FMD.

Hypertension in women

“…For example, fibromuscular dysplasia, a nonatherosclerotic noninflammatory vascular disease that can affect renal arteries and cause renovascular hypertension, primarily affects women aged from age 20 to 60, but can also affects men and children. 35 Fibromuscular dysplasia should be investigated with noninvasive tests, such as renal artery duplex ultrasound, computed tomography angiography and magnetic resonance angiography, in young women with cervical bruits or development of hypertension o35 years. Percutaneous balloon angioplasty is the preferred treatment.…”

Internal carotid artery dissection as a cause of severe ischemic stroke with lethal outcome

“…Это свидетельствует о том, что обнаруженные изменения артериальной стенки не были следстви-ем диссекции, а предшествовали ей, отражая нали-чие артериопатии, которая и предрасполагала к дис-секции. В целом изменения ВЭМ (истончение, рас-щепление, отсутствие на отдельных участках) и ме-дии (участки фиброза, очевидно, на месте погибших миоцитов, нарушение ориентации миоцитов с обра-зование «клубков», снижение числа эластических волокон, нарушение их ориентации на некоторых участках, гиперэластоз) аналогичны таковым при фиброзно-мышечной дисплазии (ФМД) [20][21][22]. Ранее мы уже отмечали это в проведенном клинико-морфологическом исследовании при диссекции [10].…”

Multicenter studies to shed light on fibromuscular displasia and cervical artery dissection

“…At least two vascular beds are involved in up to 28% of the patients. FMD is a pathological diagnosis, with medial fibroplasia being the most frequent finding, but a classical "beading" radiologic pattern with dilatations and stenoses strongly supports clinical suspicion 2,3 . Other radiological patterns such as long, smooth narrowing have also been described.…”

“…FMD is a segmental, noninflammatory, nonatherosclerotic vasculopathy that affects primarily the carotid and renal arteries, but can also involve the vertebral artery and other territories 2 . At least two vascular beds are involved in up to 28% of the patients.…”

“…It has been suggested that, even though FMD was previously considered to be rare, it may occur in 4% of adult women, being asymptomatic in most cases 2 . FMD is associated with a greater prevalence of intracranial aneurysms, as previously reported in this journal 4 , and with a greater risk of arterial dissection.…”