Management of Fibromuscular Dysplasia
Percutaneous transluminal renal angioplasty (PTRA) without stenting is the treatment of choice for symptomatic renal artery fibromuscular dysplasia, while all patients should receive medical blood pressure management and antiplatelet therapy. 1, 2
Initial Medical Management
All patients with fibromuscular dysplasia require medical therapy as the foundation of treatment, regardless of whether they undergo revascularization 1, 2:
- Start antiplatelet therapy (aspirin or clopidogrel) to prevent thromboembolic complications in all patients with FMD 3
- Initiate blood pressure control with RAS blockers (ACE inhibitors or ARBs) as first-line agents when percutaneous intervention is not immediately feasible 1, 2
- Monitor renal function carefully when using RAS blockers, particularly in bilateral stenoses or solitary functioning kidney, as acute renal failure can occur 1
- Alternative antihypertensive agents include calcium channel blockers (dihydropyridine or non-dihydropyridine) and alpha-receptor antagonists if RAS blockers are contraindicated 1
Revascularization Strategy for Renal Artery FMD
PTRA without stenting should be considered for patients with hypertension and hemodynamically significant renal artery stenosis due to FMD (Class IIa recommendation) 1, 2:
- Avoid stenting as first-line therapy; reserve stents only for dissection or balloon angioplasty failure 2, 3
- Perform procedures in experienced centers due to technical complexity and risk of complications 1
- Consider open surgical revascularization only when endovascular therapy is technically unfeasible, has failed, or for complex aneurysms and lesions involving arterial bifurcations 1, 2
The goal of renal artery intervention is to restore renal perfusion pressure and achieve blood pressure normalization or improvement 1, 4.
Cerebrovascular FMD Management
For carotid and vertebral artery involvement 3:
- Carotid angioplasty with or without stenting is reasonable for patients with retinal or hemispheric cerebral ischemic symptoms related to FMD of the ipsilateral carotid artery (Class IIa recommendation) 3
- Do not revascularize asymptomatic carotid FMD, regardless of stenosis severity (Class III recommendation) 3
- Antiplatelet therapy remains the cornerstone for cerebrovascular FMD management 4, 5
Systemic Disease Surveillance
FMD is a systemic arterial disease requiring comprehensive vascular evaluation 1, 2, 3:
- Perform CT or MRI angiography from head to pelvis at diagnosis to identify involvement in multiple vascular beds, particularly carotid, vertebral, renal, and iliac arteries 1, 2
- Annual non-invasive imaging of carotid arteries is reasonable initially to detect disease progression or new lesions (Class IIa recommendation) 2, 3
- Reduce imaging frequency once stability has been confirmed over time 2
- Screen for aneurysms as they represent a potential complication requiring surveillance or intervention 2, 5
Diagnostic Workup
- Duplex ultrasonography as initial screening (Class I recommendation) 2, 3
- CT angiography or MR angiography for comprehensive screening (Class I recommendation) 2, 3
- Catheter angiography as the gold standard when clinical suspicion is high and noninvasive tests are inconclusive 2, 3
- Do not use captopril renal scintigraphy, selective renal vein renin measurements, or plasma renin activity for screening (Class III recommendation) 2, 3
- Assess for very elevated renin levels which raise suspicion for renovascular hypertension 1, 2
Critical Pitfalls to Avoid
- Never use stents as first-line therapy for renal FMD—balloon angioplasty alone is superior 2, 3
- Never assume isolated disease—always evaluate multiple vascular beds as FMD is systemic 1, 3
- Never revascularize asymptomatic carotid lesions—medical management is appropriate 3
- Monitor renal function closely when using RAS blockers in bilateral disease to prevent acute kidney injury 1, 2
- Control blood pressure aggressively to prevent arterial dissection, which can occur in multiple vascular territories 1