Fibromuscular dysplasia: Advances in understanding and management

Abstract

Several key findings in recent years have reshaped our understanding of fibromuscular dysplasia (FMD), an uncommon nonatherosclerotic disease of medium-sized arteries that affects mainly women. While the true prevalence of this disease remains unknown, studies suggest that more people may be affected than previously reported. Better understanding of the clinical manifestations and natural history of FMD and advances in diagnostic imaging have altered the clinical approach to managing patients with this uncommon vascular disease. Although there are a multitude of unanswered questions regarding FMD, this review highlights recent insights and how this information has modified clinical care for those affected.

Hemorrhagic Retinopathy and Optic Nerve Sheath Hemorrhage Associated With Fatal Subarachnoid Hemorrhage from a Ruptured Intracranial Aneurysm Due to Segmental Fibromuscular Dysplasia

“…FMD may represent a nonspecific reaction to a disturbance of the vascular extracellular matrix (18). This inference is supported by the occurrence of FMD in a variety of disorders affecting the extracellular matrix, such as Ehlers-Danlos syndrome, a-1 antitrypsin deficiency, and aortic coarctation (1,(18)(19)(20)(21)(22).…”

A Review of Primary Vasculitis Mimickers Based on the Chapel Hill Consensus Classification

“…Fibromuscular Dysplasia. Fibromuscular dysplasia (FMD) is an idiopathic segmental nonatherosclerotic, noninflammatory arteriopathy that predominantly affects women [28,29]. Classification is based on angiographic appearance: either focal or multifocal (string of beads) [30].…”

“…The most severe complications are acute kidney injury, renal infarction, transient ischemic attack, stroke, myocardial infarction, and mesenteric ischemia. Diagnosis is usually achieved in the presence of clinical manifestations and compatible vascular imaging [28,30,32,33].…”

Renal Artery Rupture in Association With Fibromuscular Dysplasia

“…It is characterized by fibrous tissue formation within the arterial walls leading to arterial narrowing. 1 , 2 Although the prevalence of FMD remains unknown, data from asymptomatic potential kidney donors revealed that 4.4% of them had FMD. 3 , 4 FMD has been associated with various vascular events including aneurysms, artery dissections, and tortousities.…”