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Renal Medicine
Published in Paul Bentley, Ben Lovell, Memorizing Medicine, 2019
Fibromuscular dysplasia: Epi: Young women (30–40s; F:M = 4:1); smokingSite: Distal (dysplasia); right-sided or bilateral
Rheumatology
Published in Stephan Strobel, Lewis Spitz, Stephen D. Marks, Great Ormond Street Handbook of Paediatrics, 2019
Clarissa Pilkington, Kiran Nistala, Helen Lachman, Paul Brogan
Non-inflammatory large-vessel vasculopathy of congenital cause: fibromuscular dysplasia.Williams syndrome.Coarctation of the aorta.Midaortic syndrome.Ehlers–Danlos type IV.Marfan syndrome.Neurofibromatosis type 1.
Renal transplantation and renovascular hypertension
Published in J Kellogg Parsons, E James Wright, The Brady Urology Manual, 2019
Highly effective therapy for fibromuscular dysplasia: 50–60% cure rateFailure rate <10%.
Spontaneous coronary artery dissection, a commonly overlooked etiology of acute coronary syndrome
Published in Journal of Community Hospital Internal Medicine Perspectives, 2020
Ashish Kumar Roy, Moni Roy, Manajyoti Yadav, Kalyan C. Potu, Sudhir Mungee
The risk factors of SCAD have been poorly elaborated till date. Initially, SCAD was thought to be only associated with hormonal and hemodynamic changes due to pregnancy and postpartum state but risk factors now include female sex, young age, extreme emotional stress, exertion, and fibromuscular dysplasia [2,3,13]. With increasing recognition of the disease in non-pregnant healthy women, other risk factors highlighted include systemic connective tissue disorders such as Marfan’s syndrome, Ehlers-Danlos and Loeys-Dietz syndrome. Underlying connective tissue disorders in general are known risk factors of intimal wall rupture in any arterial vessel, including coronary arteries. Fibromuscular dysplasia (FMD) is now known to be associated with this condition. In one study, noncoronary FMD was seen in 86% of patient [3]. Several other authors have reported association between FMD and SCAD [19–24]. Our patient did not have a known history of FMD, or history such as uncontrolled hypertension suggestive of it. No clear recommendation to evaluate for underlying FMD in young females diagnosed with SCAD exist. Our patient therefore did not undergo further imaging study to evaluate for possible underlying FMD.
Cervical artery dissection: fibromuscular dysplasia versus vascular Ehlers–Danlos syndrome
Published in Blood Pressure, 2019
Caroline Henrard, Hendrica Belge, Sophie Fastré, Silvia Di Monaco, Nicole Revencu, Frank Hammer, Agnès Pasquet, Alexandre Persu
In the present case, the patient was referred for a suspicion of fibromuscular dysplasia. MRA and catheter-based arteriography had indeed disclosed beading suggestive of multifocal fibromuscular dysplasia. The patient is a middle-aged woman, which corresponds to the classical demographics of fibromuscular dysplasia. Fibromuscular dysplasia is thought to be at the origin of 15% of cases of cervical artery dissection, probably representing an underestimation [7, 8]. According to registries, 3–11% of patients with FMD have at least another relative with FMD, the lower figures coming from more recent publications. However, in most familial cases, clinical presentation was renal artery fibromuscular dysplasia in two or more siblings rather than multiple dissections in other arterial beds [9].
Prevalence of smoking and clinical characteristics in fibromuscular dysplasia. The ARCADIA-POL study
Published in Blood Pressure, 2019
Piotr Dobrowolski, Magdalena Januszewicz, Helena Witowicz, Ewa Warchoł-Celińska, Anna Klisiewicz, Urszula Skrzypczyńska-Banasik, Marek Kabat, Katarzyna Kowalczyk, Anna Aniszczuk-Hybiak, Elżbieta Florczak, Adam Witkowski, Andrzej Tykarski, Krystyna Widecka, Małgorzata Szczerbo-Trojanowska, Witold Śmigielski, Wojciech Drygas, Ilona Michałowska, Piotr Hoffman, Aleksander Prejbisz, Andrzej Januszewicz
Fibromuscular dysplasia (FMD) has been defined as a nonatherosclerotic, non-inflammatory, idiopathic and segmental disease of the musculature of arterial walls, leading to stenosis of small- and medium-sized arteries [9,10]. Although a variety of genetic, mechanical, and hormonal factors have been proposed, the cause of FMD is still poorly understood and its development is likely related to a combination of genetic and environmental factors [9,10]. In this group of patients, smoking was identified as a potential factor contributing to FMD in previous observational studies [3,6]. However, the association between smoking and FMD as well as the impact of smoking on the development of major vascular complications in those patients remain undefined [9,10].