What is the treatment for fibromuscular dysplasia?

Treatment of Fibromuscular Dysplasia

For patients with fibromuscular dysplasia (FMD), antiplatelet therapy with aspirin is the recommended first-line medical treatment for all symptomatic patients, while percutaneous transluminal angioplasty without stenting is the treatment of choice for patients with symptomatic renal or carotid FMD. 1

Understanding Fibromuscular Dysplasia

Fibromuscular dysplasia is a nonatherosclerotic, noninflammatory vascular disease that primarily affects medium-sized arteries, most commonly the renal and carotid arteries. It predominantly affects women between 30-50 years of age but can occur in any arterial bed 1, 2.

Treatment Algorithm

1. Medical Management (First-Line)

• Antiplatelet Therapy

  • Aspirin is recommended for all symptomatic FMD patients 1
  • Particularly beneficial in patients with carotid artery FMD to prevent thromboembolism

• Antihypertensive Therapy

  • For patients with hypertension due to renal FMD:
    • RAS blockers (ACE inhibitors or ARBs) are commonly used 1
    • Careful monitoring of renal function is required, especially in bilateral renal artery stenosis

• Lifestyle Modifications

  • Smoking cessation (critical as smoking is a risk factor)
  • Regular physical activity
  • Healthy diet 1

2. Interventional Management

• Renal Artery FMD

  • Indications for intervention:
    • Resistant hypertension despite optimal medical therapy
    • Medication intolerance
    • Impaired renal function or ischemic nephropathy 1, 2
  • Procedure of choice: Percutaneous transluminal renal angioplasty (PTRA) without stenting 1
    • Stenting is not routinely indicated as pressure gradients are normally completely abolished with angioplasty alone 2

• Cerebrovascular FMD

  • Indications for intervention:
    • Retinal or hemispheric cerebral ischemic symptoms related to ipsilateral carotid FMD 1
  • Procedure of choice: Carotid angioplasty with or without stenting

• Surgical Revascularization

  • Reserved for cases with:
    • PTRA complications (thrombosis, perforation, progressive dissection)
    • Repeated PTRA failure or restenosis 2

3. Management of Special Scenarios

• Asymptomatic FMD

  • Carotid artery: Revascularization is NOT recommended regardless of stenosis severity 1
  • Renal artery: Medical management only 1

• FMD-Related Dissection

  • Antiplatelet therapy is reasonable for patients with ischemic symptoms 1

• FMD with Aneurysms

  • Treatment with covered stent or open surgical repair 1

Surveillance and Follow-up

• Annual noninvasive imaging of affected arteries initially to detect changes in disease extent or severity 1
• More frequent monitoring in the first few years after diagnosis
• Regular blood pressure monitoring for patients with renal FMD

Important Clinical Considerations

• FMD lesions are typically truncal or distal, whereas atherosclerotic lesions are more often proximal or ostial 2

• Diagnostic imaging choice:

  • Intra-arterial digital subtraction angiography remains the gold standard, especially for younger patients and those with suspected branch lesions
  • Ultrasonography may be sufficient for older patients (>50-55 years) with suspected FMD 2

• Centralization of management is recommended due to the specialized nature of treatment 2

The natural history of FMD is relatively benign, with progression occurring in only a minority of patients 3, but proper treatment can significantly improve outcomes and quality of life for symptomatic individuals.