Table of contents
Fibromuscular dysplasia
Background
Overview
Definition
FMD is a noninflammatory, nonatherosclerotic arterial disease of medium-sized arteries characterized by arterial stenosis, occlusion, aneurysm, and dissection.
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Pathophysiology
The exact cause of FMD is unknown.
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Disease course
Clinical manifestations of FMD depend on the artery involved (commonly carotid, renal, vertebral) resulting in stenosis, occlusion, aneurysm, and dissection. Renal involvement presented with renovascular hypertension; carotid artery involvement symptoms included headache, pulsatile tinnitus, and dizziness; and mesenteric (celiac and mesenteric) artery involvement presented with abdominal pain, hypertension, diarrhea, nausea, vomiting, headache, hemoperitoneum, and shock.
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Prognosis and risk of recurrence
The in-hospital mortality associated with FMD is 0.74%.
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Guidelines
Key sources
The following summarized guidelines for the evaluation and management of fibromuscular dysplasia are prepared by our editorial team based on guidelines from the European Society of Cardiology (ESC 2024), the American Heart Association (AHA/ASA 2021), the Hypertension Canada (HC 2020), the Society for Vascular Surgery (SVS 2020), the American College of Radiology (ACR 2019), the Society for Vascular Medicine (SVM/ESH ...
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Screening and diagnosis
Indications for testing, symptomatic patients: as per HC 2020 guidelines, evaluate for FMD-related renal artery stenosis in patients with hypertension presenting with ≥ 1 of the following clinical clues:
significant (> 1.5 cm), unexplained asymmetry in kidney sizes
abdominal bruit without apparent atherosclerosis
FMD in another vascular territory
family history of FMD.
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Indications for testing (patients with RAA)
Indications for testing (family relatives)
Diagnosis
Classification and risk stratification
Diagnostic investigations
Renal artery imaging: as per HC 2020 guidelines, obtain MRA or CTA to evaluate for FMD of the renal arteries.
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Cervicocephalic vascular imaging
Peripheral artery imaging
Imaging of other arteries
Medical management
Therapeutic procedures
Renal artery revascularization: as per ESC 2024 guidelines, consider performing renal artery angioplasty without stenting in patients with hypertension and hemodynamically significant renal artery stenosis due to FMD.
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Carotid artery revascularization
Surgical interventions
Surgical revascularization: as per HC 2020 guidelines, consider performing surgical revascularization in patients with complex lesions of FMD in the renal arteries less amenable to angioplasty, stenosis associated with complex aneurysm, and restenosis despite two unsuccessful attempts of angioplasty.
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Specific circumstances
Patients with ischemic stroke: as per AHA/ASA 2021 guidelines, initiate antiplatelet therapy, control BP control, and offer lifestyle modifications for the prevention of future ischemic events in patients with a history of ischemic stroke or TIA and FMD without other attributable causes.
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Kidney donation