What is the management approach for fibromuscular dysplasia?

Management of Fibromuscular Dysplasia

Foundation: Universal Medical Therapy for All Patients

All patients with fibromuscular dysplasia require antiplatelet therapy and blood pressure control as the cornerstone of management, regardless of whether they undergo revascularization. 1, 2

• Start aspirin 81-325 mg daily immediately in all patients with FMD, including those who are completely asymptomatic 3
• Initiate blood pressure control with ACE inhibitors or ARBs as first-line agents when hypertension is present 2, 4
• Monitor renal function carefully when using RAS blockers, particularly in bilateral stenoses or solitary functioning kidney, as acute renal failure can occur 2
• Use calcium channel blockers or alpha-receptor antagonists if RAS blockers are contraindicated 2
• Continue antiplatelet therapy long-term, especially when radiographic abnormalities persist 3

Renal Artery FMD: When to Intervene

Percutaneous transluminal renal angioplasty (PTRA) without stenting is the treatment of choice for symptomatic renal artery FMD presenting with hypertension or renal impairment. 2, 3

Indications for Revascularization:

• Hypertension with hemodynamically significant renal artery stenosis 2
• Refractory hypertension despite medical management 1
• Worsening renal function 1
• Recent onset hypertension in young patients 1

Technical Approach:

• Perform balloon angioplasty alone—never use stents as first-line therapy 2, 3
• Reserve stenting only for dissection or balloon angioplasty failure 2, 4
• Refer to experienced centers due to technical complexity 2
• Consider open surgical revascularization only when endovascular therapy is technically unfeasible, has failed, or for complex aneurysms and lesions involving arterial bifurcations 2, 4

Cerebrovascular FMD: Symptom-Driven Strategy

For patients with stroke or TIA attributable to FMD, antiplatelet therapy is the primary treatment; carotid angioplasty may be reasonable for recurrent ischemic events despite optimal medical management. 1, 3

Symptomatic Carotid/Vertebral FMD:

• Initiate antiplatelet therapy for all patients with history of ischemic stroke or TIA without other attributable causes 1
• For patients with dissection and FMD but no intraluminal thrombus, antiplatelet therapy is reasonable over anticoagulation 1, 3
• Never use anticoagulation for intracranial dissections due to increased subarachnoid hemorrhage risk 3
• Consider carotid angioplasty with or without stenting for recurrent ischemic stroke without other attributable causes despite optimal medical management 1, 2

Asymptomatic Carotid FMD:

• Do not revascularize asymptomatic carotid FMD, regardless of stenosis severity 2, 3
• Continue antiplatelet therapy even in asymptomatic patients 3

Systemic Disease Surveillance: The Critical Pitfall

FMD is a systemic arterial disease requiring comprehensive vascular evaluation at diagnosis—never assume isolated disease. 2, 3

Initial Workup:

• 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 2, 4
• Screen for aneurysms as they represent a potential complication requiring surveillance or intervention 2
• Use duplex ultrasonography as initial screening 2, 4
• Catheter angiography is the gold standard when clinical suspicion is high and noninvasive tests are inconclusive 2, 4

Ongoing Surveillance:

• Perform annual non-invasive imaging of carotid arteries initially to detect disease progression or new lesions 2, 3
• Reduce imaging frequency once stability has been confirmed over time 2, 4
• Monitor blood pressure and renal function regularly in all patients with renal artery FMD 2

Tests to Avoid:

• Do not use captopril renal scintigraphy, selective renal vein renin measurements, or plasma renin activity for screening 2, 4

Key Clinical 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 2, 3
• Never revascularize asymptomatic carotid lesions—medical management is appropriate 2, 3
• Never withhold antiplatelet therapy in asymptomatic patients—guidelines clearly support its use 3
• Monitor renal function closely when using RAS blockers in bilateral disease to prevent acute kidney injury 2
• Control blood pressure aggressively to prevent arterial dissection, which can occur in multiple vascular territories 2