FIBROMUSCULAR DYSPLASIA (FMD)

• a noninflammatory, nonatherosclerotic disorder that leads to arterial stenosis,

occlusion, aneurysm, dissection, and arterial tortuosity
• most frequently involved arteries are the renal (75 to 80 percent of patients), internal
carotid, and vertebral arteries (approximately 75 percent of patients have carotid
and/or vertebral involvement), followed by visceral and external iliac arteries
• more common among females (approximately 90 percent of cases are in women
CLASSIFICATION
• virtually always diagnosed radiographically, and histology is not typically available
 • angiographic classification:
o Multifocal FMD (more common) has the angiographic appearance of a "string
of beads.
▪ corresponds pathologically to medial fibroplasia, the most common
histologic type, and to perimedial fibroplasia, which is less common.
o Focal FMD (less common) has the angiographic appearance of a
"circumferential or tubular stenosis" and corresponds pathologically to intimal
fibroplasia.
▪ Medial hyperplasia and periarterial hyperplasia are histologic types
that may also have a focal appearance.

CLINICAL FEATURES
• most common presenting signs and symptoms of FMD include
o hypertension,
o headache,
o pulsatile tinnitus,
o neck pain,
o and cervical bruit
• Other presenting signs and symptoms can include
o flank or
o abdominal pain,
o an abdominal bruit,
o transient ischemic attack,
o and stroke.
• disease manifestations may result from
o ischemia related to stenosis,
o dissection,
o and occlusion of arteries,
o rupture of aneurysms,
o or embolization of intravascular thrombi
• FMD should be suspected in the following settings :
o In a patient with hypertension (particularly in a woman under the age of 60 years)
who has findings that would prompt an evaluation for secondary hypertension or
renovascular disease
o The presence of a carotid bruit in a patient under the age of 60 years,
especially if other common risk factors for atherosclerosis, aside from
hypertension, are absent.
o Severe and persistent headache or pulsatile tinnitus that has no other obvious
explanation or is accompanied by focal neurologic symptoms or signs.
o transient ischemic attack or stroke in a woman under the age of 60 years,
especially if other common risk factors for atherosclerosis, aside from
hypertension, are absent.
o Dissection of a peripheral or coronary artery (ie, spontaneous coronary artery
dissection [SCAD]).
o Aneurysm of a visceral, carotid, vertebral, or intracranial artery.
o Aortic aneurysm in a patient under 60 years of age.
DIAGNOSIS
• diagnosis is confirmed by diagnostic imaging that reveals consistent findings
• noninvasive imaging test is usually performed first (ie, computed tomography
angiography [CTA], magnetic resonance angiography [MRA], or, in some centers
with sufficient expertise, duplex ultrasound)
• Digital subtraction angiography (DSA) is performed in patients if there is a high
clinical suspicion of FMD, and treatment with revascularization is planned if a
stenosis is found
• Negative DSA excludes a diagnosis of FMD in the vascular bed that was imaged
• every patient diagnosed with FMD should have one-time, head-to-pelvic cross-
sectional imaging. The preferred imaging strategy is CTA, with MRA as an
alternative.
• conditions that most commonly mimic the presentation of FMD are atherosclerotic
vascular disease and vasculitis
• Atherosclerosis usually involves the ostial or proximal segment of the arteries,
whereas FMD involves the middle or distal segment; the "string of beads" appearance
is also unique to FMD
• Unlike patients with a vasculitic process, those with FMD generally will not have
associated anemia, thrombocytopenia, or abnormalities of acute phase reactants (eg,
erythrocyte sedimentation rate or C-reactive protein), given that it is a
noninflammatory process
• Patients who are treated medically (rather than with revascularization) should have
monitoring of the blood pressure response to antihypertensive therapy and serum
creatinine levels every three months
• decision against revascularization may need to change if hypertension is treatment
resistant or if there is progressive loss of kidney function
• Noninvasive imaging tests, such as duplex ultrasound, should be obtained every 6 to
12 months
• Since radiation exposure is high, CTA should only be repeated if there is a strong
clinical suspicion regarding the progression of the disease
MANAGEMENT
• Options for management of hypertension in patients with FMD include
antihypertensive drug therapy and revascularization.
• Most patients with renal FMD and hypertension will require antihypertensive therapy,
even if they undergo revascularization
• initial antihypertensive drug class of choice in FMD is an angiotensin-converting
enzyme (ACE) inhibitor or angiotensin receptor blocker (ARB)
• If goal blood pressure is not reached with angiotensin inhibition alone, other
antihypertensive drugs (such as a thiazide diuretic or a long-acting calcium channel
blocker) should be added as necessary. Goal blood pressure in patients with FMD is
the same as in similarly aged hypertensive patients who have an etiology other than
FMD
• We suggest that the following patients with renal FMD and hypertension undergo
renal artery revascularization rather than antihypertensive therapy alone
 o Those with recent-onset hypertension, particularly younger patients who are
less likely to have underlying atherosclerotic disease, in whom the goal is to
cure hypertension or significantly reduce the number of antihypertensive
medications.
o Those with resistant hypertension despite compliance with an appropriate
three-drug regimen.
o Those who are unable to tolerate antihypertensive medications or who are
noncompliant with their medication regimen.
o Adults with bilateral renal FMD, or unilateral renal FMD to a single
functioning kidney, who have otherwise unexplained progressive renal
insufficiency that is thought to result from renal artery stenosis (ie, "ischemic
nephropathy"). The clinical manifestations of ischemic nephropathy are
presented elsewhere.
o Hypertensive children with renal FMD.
• In most patients with FMD who are selected for renal revascularization, we suggest
percutaneous transluminal angioplasty (PTA) rather than surgery
• surgery is preferred if angioplasty is not possible or if there is also a renal artery
aneurysm present
• PTA is typically performed without placement of stent (unlike PTA for
atherosclerotic renal artery stenosis
• stents are only placed when a dissection results from the performance of PTA or in
the rare instance in which a perforation of the renal artery occurs during angioplast
• If surgery is performed, aortorenal bypass with a saphenous vein graft is the most
commonly performed revascularization procedure in patients with renal FMD
• An important limitation of pursuing medical therapy alone is that renal artery stenosis
and kidney dysfunction may progress despite good blood pressure control.
• every patient with FMD should have periodic measurement of serum creatinine every
6 months and duplex ultrasound every 12 months.
• Patients who undergo percutaneous revascularization should have duplex
ultrasonography and serum creatinine measurements performed on the first office visit
post procedure, then every six months for two years, and then yearly if stable