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| Fibromuscular dysplasia Classification and external resources |
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| The "string-of-beads" appearance of medial fibromuscular dysplasia on angiography. | |
| ICD-10 | I77.3 |
| ICD-9 | 447.3, 447.8 |
| OMIM | 135580 |
| DiseasesDB | 30163 |
| eMedicine | neuro/432 |
| MeSH | D005352 |
Fibromuscular dysplasia (FMD), or fibromuscular dysplasia of arteries,[1] 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. Angiography with contrast will show a characteristic "string of beads" morphology in a vessel affected by FMD.
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Genetic prevalence
Fibromuscular dysplasia is an autosomal dominant[2] disorder. It tends to occur between 14 and 50 years of age, but it has also been found in children younger than age 14.
Histopathology
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. 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.
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 affected 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.citation needed
Ace Inhibitors are contraindicated in renal artery stenosis due to the risk of renal failure with inhibition of the Renin-Angiotensin-Aldosterone system to provide blood flow in the stenotic vessel.citation needed
See also
References
- ^ Online 'Mendelian Inheritance in Man' (OMIM) 135580
- ^ Rushton, Ar (Feb 1980). "The genetics of fibromuscular dysplasia". Archives of internal medicine 140 (2): 233–6. ISSN 0003-9926. PMID 7352819.
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