The European/international fibromuscular dysplasia registry and initiative (FEIRI) - Clinical phenotypes and their predictors based on a cohort of 1000 patients

Pappaccogli, M, Di Monaco, S, Warchoł-Celińska, E, Lorthioir, A, Amar, L, Aparicio, LS, Beauloye, C, Bruno, RM, Chenu, P, De Leeuw, P, De Backer, T, Delmotte, P, Dika, Z, Gordin, D, Heuten, H, Iwashima, Y, Krzesinski, JM, Kroon, AA, Mazzolai, L, Poch, E, Sarafidis, P, Seinturier, C, Spiering, W, Toubiana, L, Van Der Niepen, P, Van Twist, D, Visonà, A, Wautrecht, JC, Witowicz, H, Xu, J, Prejbisz, A, Januszewicz, A, Azizi, M, Persu, A, Hammer, F, Goffette, P, Astarci, P, Peeters, Anna, Verhelst, R, Vikkula, M, Van Tussenbroek, F, Gevaert, S, Hemelsoet, D, Defreyne, L, Yperzeele, L, Van Der Zijden, T, Lengelé, JP, Sprynger, M, Verhamme, P, Vanassche, T, Scoppettuolo, P, Vinck, W, Dobrin, V, Teodora, Y, Wang, J, Jelaković, B, Tikkanen, I, Venermo, M, Mäkelä, A, Plouin, PF, Jeunemaitre, X, Chédid, A, Mousseaux, E, Touzé, E, Ormezzano, O, Thony, F, Mahfoud, F, Kulenthiran, S, Piperidou, A, Doumas, M, Stergiou, GS, Vlahakos, D, Canning, C, Sharabi, Y, Morganti, A, Taddei, S, Romanini, C, Petrucci, I, Rabbia, F, Rossi, GP, Lerco, S, Minuz, P, Mansueto, G, De Marchi, S, Marcon, D, Salice, P, Bigolin, P, Zingaretti, V, Cianci, R, Zedde, M, Matteucci, MC, Kawarada, O, Kadoya, Y, Kroon, B, Van Den Born, BJ, Høieggen, A, Sommer, MS and Witkowski, A 2021, The European/international fibromuscular dysplasia registry and initiative (FEIRI) - Clinical phenotypes and their predictors based on a cohort of 1000 patients, Cardiovascular Research, vol. 117, no. 3, pp. 950-959, doi: 10.1093/cvr/cvaa102.

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Title The European/international fibromuscular dysplasia registry and initiative (FEIRI) - Clinical phenotypes and their predictors based on a cohort of 1000 patients
Author(s) Pappaccogli, M
Di Monaco, S
Warchoł-Celińska, E
Lorthioir, A
Amar, L
Aparicio, LS
Beauloye, C
Bruno, RM
Chenu, P
De Leeuw, P
De Backer, T
Delmotte, P
Dika, Z
Gordin, D
Heuten, H
Iwashima, Y
Krzesinski, JM
Kroon, AA
Mazzolai, L
Poch, E
Sarafidis, P
Seinturier, C
Spiering, W
Toubiana, L
Van Der Niepen, P
Van Twist, D
Visonà, A
Wautrecht, JC
Witowicz, H
Xu, J
Prejbisz, A
Januszewicz, A
Azizi, M
Persu, A
Hammer, F
Goffette, P
Astarci, P
Peeters, AnnaORCID iD for Peeters, Anna
Verhelst, R
Vikkula, M
Van Tussenbroek, F
Gevaert, S
Hemelsoet, D
Defreyne, L
Yperzeele, L
Van Der Zijden, T
Lengelé, JP
Sprynger, M
Verhamme, P
Vanassche, T
Scoppettuolo, P
Vinck, W
Dobrin, V
Teodora, Y
Wang, J
Jelaković, B
Tikkanen, I
Venermo, M
Mäkelä, A
Plouin, PF
Jeunemaitre, X
Chédid, A
Mousseaux, E
Touzé, E
Ormezzano, O
Thony, F
Mahfoud, F
Kulenthiran, S
Piperidou, A
Doumas, M
Stergiou, GS
Vlahakos, D
Canning, C
Sharabi, Y
Morganti, A
Taddei, S
Romanini, C
Petrucci, I
Rabbia, F
Rossi, GP
Lerco, S
Minuz, P
Mansueto, G
De Marchi, S
Marcon, D
Salice, P
Bigolin, P
Zingaretti, V
Cianci, R
Zedde, M
Matteucci, MC
Kawarada, O
Kadoya, Y
Kroon, B
Van Den Born, BJ
Høieggen, A
Sommer, MS
Witkowski, A
Journal name Cardiovascular Research
Volume number 117
Issue number 3
Start page 950
End page 959
Total pages 10
Publisher Oxford University Press
Place of publication Oxford, Eng.
Publication date 2021-03-01
ISSN 0008-6363
Keyword(s) Aneurysm
Fibromuscular dysplasia
Renovascular hypertension
European/International FMD Registry and Initiative (FEIRI), and the Working Group ‘Hypertension and the Kidney’ of the European Society of Hypertension (ESH)
Summary Abstract Aims 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. Methods and results 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. Conclusions 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.
Language eng
DOI 10.1093/cvr/cvaa102
Field of Research 1102 Cardiorespiratory Medicine and Haematology
HERDC Research category C1 Refereed article in a scholarly journal
Persistent URL

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