RT Journal Article
T1 The European/International Fibromuscular Dysplasia Registry and Initiative (FEIRI)-clinical phenotypes and their predictors based on a cohort of 1000 patients.
A1 Pappaccogli, Marco
A1 Di Monaco, Silvia
A1 Warchoł-Celińska, Ewa
A1 Lorthioir, Aurélien
A1 Amar, Laurence
A1 Aparicio, Lucas S
A1 Beauloye, Christophe
A1 Bruno, Rosa Maria
A1 Chenu, Patrick
A1 de Leeuw, Peter
A1 De Backer, Tine
A1 Delmotte, Philippe
A1 Dika, Zivka
A1 Gordin, Daniel
A1 Heuten, Hilde
A1 Iwashima, Yoshio
A1 Krzesinski, Jean-Marie
A1 Kroon, Abraham A
A1 Mazzolai, Lucia
A1 Poch, Esteban
A1 Sarafidis, Pantelis
A1 Seinturier, Christophe
A1 Spiering, Wilko
A1 Toubiana, Laurent
A1 Van der Niepen, Patricia
A1 van Twist, Daan
A1 Visonà, Adriana
A1 Wautrecht, Jean-Claude
A1 Witowicz, Helena
A1 Xu, Jianzhong
A1 Prejbisz, Aleksander
A1 Januszewicz, Andrzej
A1 Azizi, Michel
A1 Persu, Alexandre
A1 European/International FMD Registry and Initiative (FEIRI), and the Working Group ‘Hypertension and the Kidney’ of the European Society of Hypertension (ESH)
K1 Aneurysm
K1 Dissection
K1 Fibromuscular dysplasia
K1 Renovascular hypertension
K1 Stroke
AB Since December 2015, the European/International Fibromuscular Dysplasia (FMD) Registry enrolled 1022 patients from 22 countries. We present their characteristics according to disease subtype, age and gender, as well as predictors of widespread disease, aneurysms and dissections. All patients diagnosed with FMD (string-of-beads or focal stenosis in at least one vascular bed) based on computed tomography angiography, magnetic resonance angiography, and/or catheter-based angiography were eligible. Patients were predominantly women (82%) and Caucasians (88%). Age at diagnosis was 46 ± 16 years (12% ≥65 years old), 86% were hypertensive, 72% had multifocal, and 57% multivessel FMD. Compared to patients with multifocal FMD, patients with focal FMD were younger, more often men, had less often multivessel FMD but more revascularizations. Compared to women with FMD, men were younger, had more often focal FMD and arterial dissections. Compared to younger patients with FMD, patients ≥65 years old had more often multifocal FMD, lower estimated glomerular filtration rate and more atherosclerotic lesions. Independent predictors of multivessel FMD were age at FMD diagnosis, stroke, multifocal subtype, presence of aneurysm or dissection, and family history of FMD. Predictors of aneurysms were multivessel and multifocal FMD. Predictors of dissections were age at FMD diagnosis, male gender, stroke, and multivessel FMD. The European/International FMD Registry allowed large-scale characterization of distinct profiles of patients with FMD and, more importantly, identification of a unique set of independent predictors of widespread disease, aneurysms and dissections, paving the way for targeted screening, management, and follow-up of FMD.
YR 2021
FD 2021
LK https://hdl.handle.net/10668/26283
UL https://hdl.handle.net/10668/26283
LA en
DS RISalud
RD Apr 8, 2025