Overview
Fibromuscular dysplasia (FMD) is an idiopathic, segmental disorder affecting arterial walls, leading to stenosis primarily in small to medium-sized arteries, including coronary arteries. It often involves renal and cervical arteries but can affect visceral arteries, presenting with a diverse clinical spectrum 1.Diagnosis
Clinical Presentation: Diverse, ranging from asymptomatic to acute emergencies 1.
Diagnostic Imaging: CT angiography, MR angiography, or conventional catheter-based angiography 1.
Screening: Recommended in patients with renal artery FMD, aneurysms, or dissections, especially for visceral artery involvement 1.Management
Treatment Dependent on Clinical Picture: No specific drug classes or doses universally recommended 1.
Extrapolation from Atherosclerotic Disease: Common practice often guided by management principles from atherosclerotic vascular diseases 1.Special Populations
Renal Involvement in Solitary Kidney: Requires careful management due to limited compensatory options 2.Key Recommendations
Screen for visceral artery FMD in patients with renal artery FMD, aneurysms, or dissections (Evidence: Expert opinion) 1.
Utilize CT, MR, or catheter angiography for definitive diagnosis of FMD (Evidence: Expert opinion) 1.
Treatment strategies should be tailored to individual clinical presentations, given the low level of evidence supporting specific interventions (Evidence: Weak) 1.References
1 Van der Niepen P, van Tussenbroek F, Devos H, Debing E, Di Monaco S, Goffette P et al.. Visceral Fibromuscular Dysplasia: From asymptomatic disorder to emergency. European journal of clinical investigation 2018. link
2 Bozzani A, Moia A, Carlino M, Caldana M, Ragni F. Response to 'Fibromuscular dysplasia affecting a two-branched renal artery in a patient with a solitary kidney: case presentation'. Clinical cardiology 2013. link