Fibromuscular dysplasia
From IDWiki
Background
- Non-inflammatory, non-atherosclerotic fibroplastic disorder
- Far more common in women (90% of cases)
Clinical Manifestations
- Arteries involved:
- Renal 58%
- Carotid or vertebral 32%
- Other arteries including iliac and intracranial 10%
Renal Artery FMD
- Hypertension, especialy early-onset <35 years or drug-resistant
- Average age of onset 43 years
- Epigastric or flank bruit
- Flank pain if dissection or aneurysm
- Progression to end-stage renal disease is rare
Cerebrovascular FMD
- Headache is most common complaint, usually migrainous
- Pulsatile tinnitus
- Dizziness or lightheadedness
- Cervical bruit
- Neck pain
- TIA or stroke
- Carotid and vertebral artery dissections, presenting with headache and neck pain, cranial nerve deficits, or Horner syndrome
- Can cause cerebral aneurysms
Mesenteric FMD
- Rare, often incidental finding
- Can cause mesenteric ischemia
FMD of the extremities
- Most commonly the external iliac arteries, though can involved brachial arteries as well
- Often asymptomatic, or claudication; rarely, acute ischemia
- May have a lower abdominal or inguinal bruit
FMD of the Coronary Arteries
- Can present as an acute coronary syndrome, though rare
Differential Diagnosis
- Standing waves caused by contrast-induced spasm
- Atherosclerosis
- Vasculitis
- Segmental arterial mediolysis
Diagnosis
- CT or MR angiography (or Duplex ultrasound, if CT unavailable)
- Measures a gradient
- Previously would use captopril renal scan
Management
Renal Artery Revascularization
- Recurs in 50-60%
Indications
- Resistant hypertension despite 3-drug regimen including diuretic
- Recent-onset hypertension, with the goal of curing it
- Renal artery dissection (needs stenting)
- Renal artery aneurysm
- Branch renal artery disease and hypertension
- Preservation of renal function if severe stenosis
Chronic
- Low-dose ASA recommended for all patients
Screening
- All patients with FMD anywhere are recommended to be screened for intracranial aneurysms with CTA or MRA
- Carotid, coronary, vertebral aneurysms all possible, so likely useful to do whole-body CTA
Hypertension
- First-line is ACEi/ARB
- Can consider percutaneous transluminal angioplasty
Dissection
- LMWH or wardarin for 3 to 6 months, or antiplatelet therapy
Follow-up
- Monitor blood pressure and creatinine every three months, then annually if stable
- Duplex ultrasound or other noninvasive imaging of the involved arteries every 6 to 12 months
Further Reading
- Olin et al. Fibromuscular Dysplasia: State of the Science and Critical Unanswered Questions. Circulation 2014;129:1048-1078.
