What is the optimal management approach for a young to middle-aged woman with fibromuscular dysplasia, likely presenting with hypertension and potential kidney disease?

Optimal Management of Fibromuscular Dysplasia

Foundation: Universal Medical Therapy for All Patients

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

• Start aspirin 81-325 mg daily immediately upon diagnosis to prevent thromboembolic complications 1, 2
• Initiate ACE inhibitors or ARBs as first-line antihypertensive agents when percutaneous intervention is not immediately feasible 3, 1
• Monitor renal function carefully when using ACE inhibitors/ARBs, particularly in bilateral stenoses or solitary functioning kidney, as acute renal failure can occur 3, 1
• Alternative antihypertensive agents include calcium channel blockers and alpha-receptor antagonists if RAS blockers are contraindicated 1

Comprehensive Diagnostic Workup

FMD is a systemic arterial disease requiring head-to-pelvis vascular evaluation at diagnosis, not just assessment of the symptomatic territory. 1, 4

• Perform duplex ultrasonography as the initial screening test (Class I recommendation) 1, 4
• Obtain CT or MRI angiography from head to pelvis to identify involvement in multiple vascular beds, particularly carotid, vertebral, renal, and iliac arteries 1, 4
• Reserve catheter angiography as the gold standard when clinical suspicion is high and noninvasive tests are inconclusive, or when proceeding directly to intervention 1, 4
• Do NOT use captopril renal scintigraphy, selective renal vein renin measurements, or plasma renin activity for screening (Class III recommendation) 1, 4
• Obtain morning plasma aldosterone and plasma renin activity to screen for primary aldosteronism, as resistant hypertension is common in FMD 1

Renal Artery FMD: Revascularization Strategy

Percutaneous transluminal renal angioplasty (PTRA) WITHOUT stenting is the treatment of choice for symptomatic renal artery FMD with renovascular hypertension or declining renal function (Class IIa recommendation). 3, 1

Indications for revascularization:

• Refractory hypertension despite medical management 1
• Worsening renal function 1
• Recent onset hypertension in a young patient with audible bruit 1, 2
• Signs of organ ischemia 3

Technical approach:

• Balloon angioplasty alone is superior to stenting as first-line therapy 3, 1
• Reserve stenting ONLY for dissection or balloon angioplasty failure 3, 1
• Perform procedures in experienced centers due to technical complexity 1
• Open surgical revascularization should be considered only for complex aneurysms, lesions involving arterial bifurcations/branches, or failed endovascular therapy 3, 1

Outcomes data:

• Endovascular therapy has lower major complication rates (6.3% vs 15.4%) and mortality (0.9% vs 1.2%) compared to open surgery 3

Cerebrovascular FMD: Symptom-Driven Management

For carotid/vertebral FMD, antiplatelet therapy is the primary treatment; revascularization is reserved for symptomatic patients with recurrent ischemic events despite optimal medical management. 3, 1

• Do NOT revascularize asymptomatic carotid FMD, regardless of stenosis severity (Class III recommendation) 1
• For patients with stroke or TIA attributable to FMD, continue antiplatelet therapy as primary treatment 1
• Carotid angioplasty with or without stenting is reasonable for recurrent ischemic symptoms despite optimal medical management (Class IIa recommendation) 3, 1
• Clinical manifestations may include stroke, TIA, carotid dissection, Horner syndrome, cranial nerve palsies, or subarachnoid hemorrhage 3

Long-Term Surveillance Strategy

Annual non-invasive imaging of carotid arteries is reasonable initially to detect disease progression or new lesions, with reduced frequency once stability is confirmed (Class IIa recommendation). 1, 4

• Perform annual carotid imaging initially to detect changes in disease extent or severity 1, 4
• Reduce imaging frequency once stability has been confirmed over time 1, 4
• Screen for aneurysms as they represent a potential complication requiring surveillance or intervention 1
• Regular blood pressure monitoring and renal function assessment in all patients with renal artery FMD 1, 2
• Continue antiplatelet therapy long-term, especially when radiographic abnormalities persist 2

Critical Pitfalls to Avoid

• Never use stents as first-line therapy for renal FMD—balloon angioplasty alone is superior 3, 1
• Never assume isolated disease—always evaluate multiple vascular beds as FMD is systemic 1, 4
• Never revascularize asymptomatic carotid lesions—medical management is appropriate 1
• Monitor renal function closely when using ACE inhibitors/ARBs in bilateral disease to prevent acute kidney injury 3, 1
• Control blood pressure aggressively to prevent arterial dissection, which can occur in multiple vascular territories 1

Special Considerations for Young Women

• FMD predominantly affects middle-aged women with a 9:1 female-to-male ratio 3
• The disease appears familial in 10% of cases, warranting family screening 5, 6
• Patients face elevated cardiovascular risk: 13.4% experience TIA, 12% experience cervical artery dissection, and 9.8% have had a stroke 4, 2
• The "string-of-beads" appearance on angiography is pathognomonic of medial FMD, accounting for 70-95% of cases 7
• Refer to specialized centers for dedicated multidisciplinary FMD management 1, 6