Fibromuscular dysplasia: Difference between revisions
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==Pathophysiology== | ==Pathophysiology== | ||
Fibromuscular dysplasia is characterized by fibrous thickening of the [[tunica intima|intima]], [[tunica media|media]], or [[adventitia]] of the [[renal artery]]. Up to 75% of all patients with FMD will have disease in the renal arteries | Fibromuscular dysplasia is characterized by fibrous thickening of the [[tunica intima|intima]], [[tunica media|media]], or [[adventitia]] of the [[renal artery]]. Up to 75% of all patients with FMD will have disease in the renal arteries. The lesions cause narrowing of the artery [[lumen (anatomy)|lumen]]. The second most common artery affected is the [[carotid]] artery, which is found in the neck and supplies the brain with blood. Less commonly, FMD affects the arteries in the abdomen (supplying the [[liver]], [[spleen]] and [[intestines]]) and extremities (legs and arms). More than one artery may have evidence of FMD in 28% of people with this disease (Luscher, 1986). All arteries should be checked if found. | ||
==Signs and Symptoms== | ==Signs and Symptoms== | ||
Revision as of 12:48, 26 April 2012
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Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor-in-Chief: Shantanu Sinha, MD,FACC
Overview
Fibromuscular dysplasia (FMD) is a disease that can cause stenosis of the renal arteries, carotid arteries, and less commonly, the arteries of the abdomen. The disease can cause hypertension, strokes, and arterial aneurysm and dissection.
In individuals with FMD, the walls of one or more arteries undergo dysplasia. Due to this abnormal cellular development, the vessels may become stenosed. A sufficient decrease in blood flow through the artery can cause symptoms. However, FMD is often diagnosed incidentally in the absence of any signs or symptoms during an imaging study.
Epidemiology and Demographics
Age
Fibromuscular dysplasia tends to occur in females between 14 and 50 years of age. However, it has been found in children under the age of 14.
Sex
Fibromuscular dysplasia affects females more than males.
Pathophysiology
Fibromuscular dysplasia is characterized by fibrous thickening of the intima, media, or adventitia of the renal artery. Up to 75% of all patients with FMD will have disease in the renal arteries. The lesions cause narrowing of the artery lumen. The second most common artery affected is the carotid artery, which is found in the neck and supplies the brain with blood. Less commonly, FMD affects the arteries in the abdomen (supplying the liver, spleen and intestines) and extremities (legs and arms). More than one artery may have evidence of FMD in 28% of people with this disease (Luscher, 1986). All arteries should be checked if found.
Signs and Symptoms
As a result of renal artery stenosis, the kidney's afferent arteriolar pressure falls. The renin-angiotensin system is activated, causing fluid retention and hypertension. Symptoms of craniocervical involvement include headaches and lightheadedness, although patients are often asymptomatic. On physical examination, one may detect neurological symptoms secondary to a stroke, a bruit over an affected artery, and diminished distal pulses.
Angiography
Angiography with contrast will show a characteristic "string of beads" morphology in a vessel affected by FMD.
Treatment
ACE inhibitors, and Angiotensin II receptor antagonists are often initial treatments of fibromuscular dysplasia. Refractory cases are often treated by balloon angioplasty or stenting of the renal artery. Besides high blood pressure control, anti-platelet drugs and blood thinner drugs may be used. Bypass surgery is a considered treatment and also vein replacement.
See also