Fibromuscular dysplasia is a non-atherosclerotic, noninflammatory vascular
disease that primary involves medium-sized and small arteries, most commonl
y the renal and carotid arteries. Dysplasic stenoses can be classified by a
ngiography into three main subtypes, multifocal (multiple contiguous stenos
es with the "string of beads" appearance), unifocal (single stenosis in a g
iven renal artery), or tubular The multifocal subtype is the most frequent
and is usually associated with medial dysplasia, whereas unifocal and tubul
ar stenoses are associated with intimal and perimedial dysplasia, respectiv
ely. Renovascular hypertension, mainly in women aged 30 to 50 years, is the
most common manifestation of renal artery fibromuscular dysplasia. Its pre
valence in hypertensive patients is estimated to less than 1 percent. The t
rue prevalence of the disease is probably higher, however, because many cas
es can go undetected in normotensive or asymptomatic hypertensive patients.
The first line treatment is percutaneous transluminal angioplasty that usu
ally allows blood pressure improvement or normalization. Stenosis progressi
on is slow and rarely leads to ischemic renal failure. Recognition of renal
artery fibromuscular dysplasia should lead to screening for associated car
otid artery lesions. Fibromuscular dysplasia can be a familial disease.