Fibromuscular dysplasia

A vascular disease affecting the arteries

Fibromuscular dysplasia
Synonyms	FMD
Pronounce
Field	Vascular medicine, Nephrology, Neurology
Symptoms	Hypertension, headache, dizziness, tinnitus, transient ischemic attack, stroke, neck pain
Complications	Arterial dissection, aneurysm, renal artery stenosis, stroke
Onset	Usually early to middle adulthood
Duration	Chronic
Types	Multifocal (string-of-beads appearance), unifocal (focal stenosis)
Causes	Unknown (idiopathic); possibly genetic and hormonal factors
Risks	Female sex, family history, smoking
Diagnosis	CT angiography, MR angiography, catheter-based angiography
Differential diagnosis	Atherosclerosis, vasculitis, Takayasu arteritis, renal artery stenosis
Prevention	No specific prevention; managing risk factors may help
Treatment	Angioplasty, antihypertensive drugs, antiplatelet drugs
Medication	ACE inhibitors, calcium channel blockers, aspirin (for stroke prevention)
Prognosis	Generally good with treatment; may require lifelong monitoring
Frequency	Rare; estimated in up to 4% of the population, more common in women
Deaths	Rare, often related to stroke or arterial rupture

Fibromuscular dysplasia (FMD) is a rare vascular condition characterized by abnormal cell growth in the walls of medium-sized arteries, leading to stenosis, aneurysm, or dissection. It most commonly affects the renal arteries and the carotid arteries, but can involve almost any arterial bed.

Pathophysiology

FMD is a non-atherosclerotic, non-inflammatory disease of the blood vessels. The abnormal growth of cells in the arterial wall leads to a "string of beads" appearance on angiography, which is most commonly seen in the renal arteries. This beading is due to alternating stenosis and aneurysms. The exact cause of FMD is unknown, but it is believed to involve genetic, hormonal, and environmental factors.

Clinical Presentation

The symptoms of FMD vary depending on the arteries affected. In the case of renal artery involvement, patients may present with hypertension due to renal artery stenosis. Carotid or vertebral artery involvement can lead to symptoms such as headaches, dizziness, or even transient ischemic attacks and stroke. Other symptoms may include abdominal pain, flank pain, or pulsatile tinnitus.

Diagnosis

FMD is diagnosed through imaging studies. Doppler ultrasound, computed tomography angiography (CTA), and magnetic resonance angiography (MRA) are commonly used to visualize the characteristic "string of beads" appearance. In some cases, a catheter angiography may be performed for a more detailed view.

Treatment

The treatment of FMD depends on the severity and location of the disease. For patients with hypertension due to renal artery stenosis, angioplasty may be performed to improve blood flow. In some cases, antihypertensive medications are used to manage blood pressure. Surgical intervention may be necessary for aneurysms or dissections. Regular monitoring and follow-up imaging are important to assess the progression of the disease.

Prognosis

The prognosis for patients with FMD varies. Many patients can manage their symptoms with medication and lifestyle changes. However, the risk of complications such as stroke or aneurysm rupture necessitates careful monitoring. Early diagnosis and treatment are crucial for improving outcomes.

Epidemiology

FMD is more common in women than in men, with a female-to-male ratio of approximately 9:1. It can occur at any age but is most often diagnosed in individuals between the ages of 30 and 50. The prevalence of FMD is not well established, as many cases may be asymptomatic and go undiagnosed.

Related pages

• Hypertension
• Aneurysm
• Stroke
• Renal artery stenosis

External links

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