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Fibromuscular dysplasia is a nonatherosclerotic, under-recognized disorder primarily seen in middle-aged women. It can lead to several complications, such as hypertension, headaches, dissections, aneurysms, myocardial infarctions, and cerebrovascular accidents, to name a few. This article provides a comprehensive review of current literature on epidemiology, etiology, diagnosis, treatment, and long-term surveillance and fibromuscular dysplasia management. In addition, it renders the role of education and prevention for patients living with this condition and family screening. Lastly, it emphasizes the importance of a comprehensive multidisciplinary care model and patient input, given the complexity of this disease and its systemic presence and protean manifestations., (Copyright © 2021 Elsevier Inc. All rights reserved.)

Fibromuscular dysplasia (FMD), a non-inflammatory arterial disease, may lead to renovascular hypertension (HTN) and cerebrovascular disease. Little is known about medication use in FMD. Clinical features and medication use were reviewed in a national FMD registry (12 US sites). Medication usage was assessed in raw and adjusted analyses. Covariates included demographic characteristics, co-morbid conditions and vascular bed involvement. A total of 874 subjects (93.6% female) were included in the analysis. Mean age was 55.6±13.1 years, 74.5% had HTN, 25.4% had a history of transient ischemic attack or stroke, and 7.5% had a history of coronary artery disease (CAD). Renal and cerebrovascular arteries were affected in 70.4% and 74.7%, respectively. Anti-platelet agents were administered to 72.9% of patients. In multivariate analyses, factors associated with a greater likelihood of anti-platelet agent use were older age (OR=1.02 per year, p=0.005), CAD (OR=3.76, p=0.015), cerebrovascular artery FMD involvement in isolation (OR=2.31, p<0.0001) or a history of previous intervention for FMD (OR=1.52, p=0.036). A greater number of anti-HTN medications was evident in isolated renal versus isolated cerebrovascular FMD patients. Factors associated with a greater number of anti-HTN medications were older age (OR=1.03 per year, p<0.0001), history of HTN (OR=24.04, p<0.0001), history of CAD (OR=2.71, p=0.0008) and a history of a previous therapeutic procedure (OR=1.72, p=0.001). In conclusion, in FMD, medication use varies based on vascular bed involvement. Isolated renal FMD patients receive more anti-HTN agents and there is greater anti-platelet agent use among patients with cerebrovascular FMD. Further studies correlating medication use in FMD with clinically meaningful patient outcomes are necessary., (© The Author(s) 2015.)

Purpose: To examine if the outcomes after endovascular treatment in hypertensive patients with renal artery fibromuscular dysplasia (FMD) and incidental atherosclerotic renal artery stenosis (ARAS) differ from the outcomes in patients with FMD alone., Materials and Methods: All cases of patients with renal artery FMD undergoing percutaneous transluminal angioplasty during the period 2002-2012 were reviewed. The patients with complete data before and after the procedure were identified (N = 84). Based on the procedural reports, these patients were separated into two cohorts: patients with isolated FMD (n = 59) and patients with concomitant atherosclerotic renal artery stenosis and FMD (ARAS-FMD) (n = 25). The medical record of each patient was reviewed for baseline blood pressure, antihypertensive medication use, and renal function data and the same data after the procedure. Procedural details including the angiographic findings, the number of stents placed, the average number of revascularization procedures, and the number of patients requiring more than one revascularization procedure were noted., Results: The study population included 68 patients (FMD, n = 46; ARAS-FMD, n = 22). Patients in the FMD and ARAS-FMD cohorts experienced comparable significant decreases in systolic and mean arterial pressures after endovascular intervention. There was no change in the number of antihypertensive medications after the procedure within or between groups. Patients in the ARAS-FMD cohort had lower baseline estimated glomerular filtration rates (P = .007); however, renal function stabilized in both groups after endovascular therapy., Conclusions: Patients with ARAS-FMD respond to endovascular therapy with outcomes similar to patients with isolated renal artery FMD., (Copyright © 2015 SIR. Published by Elsevier Inc. All rights reserved.)

Renal artery fibromuscular dysplasia (FMD) may be underdiagnosed. We evaluated the prevalence of FMD in CORAL (Cardiovascular Outcomes in Renal Atherosclerotic Lesions) renal artery stent trial participants, in which FMD was an exclusion criterion for inclusion. We also evaluated the prevalence of FMD in a relatively healthy population of patients undergoing computed tomographic angiographic (CTA) screening for renal donor evaluation. All renal donor CTAs performed at our institution from January 2003 through November 2011 were retrospectively reviewed for the presence of FMD along with patient sex and age. These results were compared to angiographic core lab (ACL) findings for the CORAL trial. The CORAL ACL database contained 997 patients (mean age 69.3 years; 50% female). Fifty-eight (5.8%) CORAL trial patients (mean age 71.8 years; 75.9% female) demonstrated incidental FMD. The renal donor cohort included 220 patients (mean age 40.5 years; 64.5% female). Five (2.3%) demonstrated FMD (mean age 48.6 years; all female). The odds of FMD in the CORAL cohort were 2.65 times that seen in the renal donor cohort (95% CI: 1.12, 7.57). In C: onclusion, the 5.8% prevalence of renal artery FMD in the CORAL trial population, the presence of which was biased against, suggests underdiagnosis., (© The Author(s) 2014.)

Background: Fibromuscular dysplasia (FMD), a noninflammatory disease of medium-size arteries, may lead to stenosis, occlusion, dissection, and/or aneurysm. There has been little progress in understanding the epidemiology, pathogenesis, and outcomes since its first description in 1938., Methods and Results: Clinical features, presenting symptoms, and vascular events are reviewed for the first 447 patients enrolled in a national FMD registry from 9 US sites. Vascular beds were imaged selectively based on clinical presentation and local practice. The majority of patients were female (91%) with a mean age at diagnosis of 51.9 (SD 13.4 years; range, 5-83 years). Hypertension, headache, and pulsatile tinnitus were the most common presenting symptoms of the disease. Self-reported family history of stroke (53.5%), aneurysm (23.5%), and sudden death (19.8%) were common, but FMD in first- or second-degree relatives was reported only in 7.3%. FMD was identified in the renal artery in 294 patients, extracranial carotid arteries in 251 patients, and vertebral arteries in 82 patients. A past or presenting history of vascular events were common: 19.2% of patients had a transient ischemic attack or stroke, 19.7% had experienced arterial dissection(s), and 17% of patients had an aneurysm(s). The most frequent indications for therapy were hypertension, aneurysm, and dissection., Conclusions: In this registry, FMD occurred primarily in middle-aged women, although it presents across the lifespan. Cerebrovascular FMD occurred as frequently as renal FMD. Although a significant proportion of FMD patients may present with a serious vascular event, many present with nonspecific symptoms and a subsequent delay in diagnosis.

Fibromuscular dysplasia is a nonatherosclerotic and noninflammatory disease that can result in stenoses of the renal arteries and hypertension, most commonly affecting middle-aged women. Percutaneous transluminal angioplasty has long been considered the mainstay of therapy and offers high rates of improved or cured hypertension. The disease involves the mid and distal renal arteries and branchpoints and poses endovascular treatment challenges that separate fibromuscular dysplasia from atherosclerotic disease. The development of smaller balloon dilation systems offers safe and highly effective endovascular treatment options for technically difficult lesions. Newer technologies such as cutting balloons also add to the armamentarium of treatment choices, which may be useful in the setting of resistant stenoses. This article focuses on the modern technical considerations in the diagnostic evaluation and endovascular treatment of renal artery fibromuscular dysplasia., (Copyright 2010 Elsevier Inc. All rights reserved.)