What is the management approach for fibromuscular dysplasia?

Management of Fibromuscular Dysplasia

Fibromuscular dysplasia (FMD) management should focus on antiplatelet therapy for all patients, with interventional procedures reserved only for symptomatic cases. 1

Understanding FMD

Fibromuscular dysplasia is a nonatherosclerotic, noninflammatory vascular disease affecting medium-sized arteries, most commonly the renal and carotid arteries, but potentially involving almost any arterial bed. It predominantly affects women between 30-50 years of age 1.

Medical Management

Antiplatelet Therapy

• First-line therapy for all FMD patients: Aspirin 81-325 mg daily to prevent thromboembolism 2, 1
• For patients with carotid or vertebral FMD, antiplatelet therapy represents the cornerstone of treatment 3

Hypertension Management

• For patients with hypertension due to renal FMD:
  • ACE inhibitors or ARBs are commonly used but require careful monitoring of renal function, especially in bilateral renal artery stenosis 1
  • Close monitoring is essential when initiating these medications

Anticoagulation for Dissection

• For patients with cervical artery dissection related to FMD:
  • Initial anticoagulation with IV heparin or LMWH followed by warfarin (target INR 2.0-3.0) for 3-6 months
  • After 3-6 months, transition to antiplatelet therapy 2, 1

Interventional Management

Renal Artery FMD

• Indication: Hypertension due to renal FMD, resistant hypertension, medication intolerance, or deteriorating renal function 2, 1
• Procedure of choice: Balloon angioplasty without stenting 2
• Surgical reconstruction is indicated for complex disease extending into segmental arteries or those with macroaneurysms 2

Cerebrovascular FMD

• For symptomatic patients only: Carotid angioplasty with or without stenting is reasonable for patients with retinal or hemispheric cerebral ischemic symptoms related to ipsilateral carotid FMD 2, 1
• Important: Revascularization is NOT recommended for asymptomatic carotid FMD regardless of stenosis severity 2, 1

Surveillance and Follow-up

• Annual non-invasive imaging (initially) of affected arteries to detect changes in disease extent or severity 2, 1
• Studies may be repeated less frequently once stability has been confirmed 2
• Regular clinical follow-up to monitor:
  • Blood pressure control
  • New symptoms
  • Disease progression
  • Medication efficacy and side effects

Special Considerations

Pitfalls to Avoid

• Don't stent renal FMD: Balloon angioplasty alone is the preferred approach for renal FMD 2
• Don't intervene on asymptomatic carotid FMD: Medical management with antiplatelet therapy is the standard of care 2, 4
• Don't miss concomitant aneurysms: FMD is associated with a higher prevalence of intracranial aneurysms, requiring appropriate screening

High-Risk Populations

• Pregnant women with FMD require referral to maternal-fetal medicine specialists 1
• Pediatric FMD patients should be referred to pediatric specialists with FMD expertise 1

Management Algorithm

1. Diagnosis confirmation: Contrast-enhanced CTA, MRA, or catheter-based angiography
2. For all patients: Initiate antiplatelet therapy (aspirin 81-325 mg daily)
3. For hypertensive patients: Add appropriate antihypertensive medications
4. For symptomatic renal FMD: Consider balloon angioplasty
5. For symptomatic cerebrovascular FMD: Consider angioplasty with or without stenting
6. For all patients: Annual imaging surveillance initially, then less frequently once stability is confirmed
7. For patients with dissection: Anticoagulation for 3-6 months followed by antiplatelet therapy

Long-term outcomes for cerebrovascular FMD are generally favorable with conservative management, with studies showing low rates of recurrent symptoms or disease progression 4.