High Prevalence of Multiple Arterial Bed Lesions in Patients With Fibromuscular Dysplasia

Plouin, Pierre‐François; Baguet, Jean-Philippe; Thony, F.; Ormezzano, Olivier; Azarine, Arshid; Silhol, F.; Oppenheim, Catherine; Bouhanick, Béatrice; Boyer, L.; Persu, Alexandre; Hammer, Frank; Gosse, Philippe; Mounier‐Véhier, Claire; Hello, C. Le; Jeunemaı̂tre, Xavier; Azizi, Michel; Amar, Laurence; Châtellier, Gilles; Mousseaux, Élie; Touzé, Emmanuel

doi:10.1161/hypertensionaha.117.09539

Cited by 127 publications

(168 citation statements)

References 23 publications

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“…Despite high rates of aneurysm and/or dissection in patients with FMD [5,9,25,26], neurovascular events occurring after FMD diagnosis were rare (2%) and most often due to spontaneous carotid artery dissection in patients with a history of previous arterial dissection (2 of 3 patients). This suggests that patients with FMD experience a similar rate of new CeAD (1% per year) as compared to patients with spontaneous CeAD in the absence of confirmed FMD [13,29].…”

Section: Discussionmentioning

confidence: 99%

“…Additionally, Plouin and colleagues prospectively evaluated 469 patients from the ARCADIA FMD registry and found that 66.3% had involvement of more than one vascular bed if aneurysm and/or dissection were defined as additional FMD sites independent of focal/multifocal findings [25]. The current data support recent recommendations to screen all FMD patients with one-time head-to-pelvis cross-sectional imaging (CTA or MRA) [5,23,25,26]. New cervical artery FMD or a change in the appearance of baseline cervical artery FMD on subsequent imaging was not observed in any patient in the current series.…”

Section: Discussionmentioning

confidence: 99%

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Natural History of Cervical Artery Fibromuscular Dysplasia and Associated Neurovascular Events

Kadian-Dodov¹,

Goldfinger²,

Gustavson³

et al. 2018

Cerebrovasc Dis

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Background and Purpose: Fibromuscular dysplasia (FMD) is a non-atherosclerotic arteriopathy most often affecting the carotid and renal arteries. In the United States Registry for FMD, 41.7% of patients experienced an aneurysm and/or dissection by the time of entry into the Registry. We sought to determine the occurrence of neurovascular events after FMD diagnosis and any changes on cervical artery imaging that may be attributable to FMD. Methods: Patients followed at the Mount Sinai Medical Center (US Registry for FMD enrollment center) with confirmed FMD and > 1 cervical artery imaging study (at least ± 6 months from the baseline carotid duplex ultrasound [CDU]) between the years 2003 and 2015 were included. Medical records and cervical artery imaging ([CDU], magnetic resonance angiogram [MRA], and computed tomography angiogram [CTA]) were reviewed. New arterial dissection, aneurysm, transient ischemic attack, stroke, or new FMD findings were recorded. Results: Among 146 FMD patients with complete information, 52 (35.6%) had an aneurysm and 52 (35.6%) had a dissection. Mean clinical follow-up was 35.3 ± 25.3 months (range 5–153 months); patients underwent 4 ± 2.7 CDU (range 1–17); 86.3% had ≥1 neck MRA or CTA. After FMD diagnosis, 3 patients (2%) experienced a new carotid artery dissection; 1 patient experienced a stroke due to concomitant atherosclerosis. No new aneurysms occurred. In patients with cervical artery FMD, imaging findings remained stable throughout follow-up. No patient developed new cervical artery FMD findings on follow-up imaging. Conclusions: No new cervical artery FMD or aneurysm was observed on subsequent imaging. New carotid dissection was uncommon over a mean follow-up period of 35.3 ± 25.3 months and was the only non-atherosclerotic vascular event observed after FMD diagnosis.

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Section: Discussionmentioning

confidence: 99%

Section: Discussionmentioning

confidence: 99%

Natural History of Cervical Artery Fibromuscular Dysplasia and Associated Neurovascular Events

Kadian-Dodov¹,

Goldfinger²,

Gustavson³

et al. 2018

Cerebrovasc Dis

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“…Overall, our data suggest that the prevalence of renal FMD in young hypertensive women has been substantially underestimated, that limiting screening to patients ≤ 30 years old when the mean age at diagnosis in current series and registries is over 50 years old will lead to miss a substantial number of cases and finally that renal duplex has not enough sensitivity to detect all cases of renal FMD. In particular, in 2 out of 5 newly diagnosed patients with FMD reported in this study, renal duplex failed to detect FMD‐related lesions.…”

Section: Discussionmentioning

confidence: 85%

“…In the European expert Consensus, screening for renal FMD is recommended in hypertensive patients, aged under 30 years, especially in women. However, in the first report from the US FMD registry, the mean age at diagnosis of FMD was 51.9 years, in the ARCADIA study it was 53 years and in the European/International registry it is currently 45.8 years . Along the same lines, the mean age at hypertension diagnosis is 43.1 years in the US registry and 36.5 years in the European/International registry, which is substantially higher than the 30 years old cut‐off proposed in the European consensus .…”

Section: Introductionmentioning

confidence: 89%

Prevalence and characteristics of renal artery fibromuscular dysplasia in hypertensive women below 50 years old

Monaco

Lengelé

Heenaye

et al. 2019

Eur J Clin Investigation

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Background Renal fibromuscular dysplasia (FMD) is typically diagnosed in young hypertensive women. The 2014 European FMD Consensus recommended screening in all hypertensive women <30 yo. However, the prevalence of renal FMD in young/middle‐aged hypertensive women remains unclear. The aim of this work was to assess the prevalence and characteristics of renal FMD in hypertensive women ≤50 yo. Methods We retrospectively included all consecutive women aged ≤50 years referred to our Hypertension Unit from 2014 to 2017 and collected standardized information on patient characteristics and screening modalities. Results Of 1083 incident hypertensive patients, 157 patients fitted with inclusion criteria. The prevalence of renal FMD varied between 3.2% in the whole sample and 7.5% in patients explored by CTA and/or MRA (n = 67). In the subgroup of patients ≤30 yo (n = 32), the corresponding figures were 3.1% and 5.6%. The yearly prevalence of FMD tended to increase over time, in parallel with increased use of CTA/MRA as a first‐line imaging modality. Out of 5 patients with renal FMD, 2 were revascularized and 1 had extra‐renal FMD. Conclusions The prevalence of renal FMD in young/middle‐aged hypertensive women is probably one order of magnitude higher than previously assumed, in the range of 3%‐8%, depending on imaging modalities. While the diagnosis of FMD does not influence short‐term management in all patients, it may allow close monitoring and prevention of complications of the disease over time. This analysis provides the rationale for a prospective, multicentre study aiming at determining the cost‐effectiveness of systematic screening for renal FMD.

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“…The mainstay of therapy is identification of ILAA using non-invasive neuroimaging, and mediation of various risk factors such as hyperlipidemia and diabetes. Currently, the presence of ILAA is presumed by the presence of focal luminal narrowing on lumen-based imaging such as brain CTA or MRA or by identifying the presence of arterial calcifications on brain CT. 3 However, not all brain arterial stenosis are atherosclerotic, as in cases of fibromuscular dysplasia or large artery vasculitis, 4, 5 . Further, many atherosclerotic plaques have only modest degrees of luminal stenosis.…”

Section: Introductionmentioning

confidence: 99%

Pathological correlates of brain arterial calcifications

Shapiro

Goldman

Morgello

et al. 2019

Cardiovascular Pathology

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Background: In clinical practice, calcifications seen on CT studies within the large brain arteries are often referred as a surrogate marker for cholesterol-mediated atherosclerosis. However, limited data exists to support the association between calcification and atherosclerosis. In this study, we examined if intracranial arterial calcifications were associated with cholesterol-mediated intracranial large artery atherosclerosis (ILAA) within the arteries of the Circle of Willis in an autopsy based sample. Methods: We carried out a cross-sectional analysis of histopathological characteristics of brain large arteries obtained from autopsy cases. Brain large arteries were examined for evidences of calcifications, which were rated as macroscopic (coalescent) and microscopic (scattered). In addition to calcification, we also obtained measurement of the arterial wall and the presence of ILAA and non-atherosclerotic arterial fibrosis. We built hierarchical models adjusted for demographic and vascular risk factors to assess the relationship between calcification and ILAA. Results: In univariate analysis, the presence of any arterial calcifications was associated with cerebral infarcts (29% v. 14%, p<0.01). Multi-variate analysis revealed that among all calcifications, coalescent calcifications were not associated with ILAA. In contrast, scattered calcifications were associated with ILAA (p<0.001), decreased lumen diameter (−1.87 +/− 0.41 mm p≤0.001) and increased luminal stenosis (0.03 +/− 0.01% p≤0.006). These findings were independent of age, sex or other vascular risk factors. Conclusions: This study demonstrates that coalescent calcifications in brain large arteries, although associated with morbidity, are not synonymous with cholesterol driven ILAA. Understanding the precise pathological components of cerebrovascular disease, including non-atherosclerotic arterial calcifications, will help develop individualized therapies beyond amelioration of traditional risk factors such as hyperlipidemia.

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High Prevalence of Multiple Arterial Bed Lesions in Patients With Fibromuscular Dysplasia

Cited by 127 publications

References 23 publications

Natural History of Cervical Artery Fibromuscular Dysplasia and Associated Neurovascular Events

Natural History of Cervical Artery Fibromuscular Dysplasia and Associated Neurovascular Events

Prevalence and characteristics of renal artery fibromuscular dysplasia in hypertensive women below 50 years old

Pathological correlates of brain arterial calcifications

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