The European/International Fibromuscular Dysplasia Registry and Initiative (FEIRI)-clinical phenotypes and their predictors based on a cohort of 1000 patients
Por:
Pappaccogli, M, Di Monaco, S, Warchol-Celinska, 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, Visona, A, Wautrecht, JC, Witowicz, H, Xu, JZ, Prejbisz, A, Januszewicz, A, Azizi, M, Persu, A, De la Llama P.F., Barros-Membrilla A.J., European Int FMD Registry Initiat, European Soc Hypertension ESH
Publicada:
1 mar 2021
Resumen:
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.
Filiaciones:
Pappaccogli, M:
Univ Turin, Dept Med Sci, Div Internal Med, Turin, Italy
Univ Turin, Dept Med Sci, Hypertens Unit, Turin, Italy
Catholic Univ Louvain, Div Cardiol, Clin Univ St Luc, 10 Ave Hippocrate, B-1200 Brussels, Belgium
Catholic Univ Louvain, Inst Rech Expt & Clin, Pole Cardiovasc Res, B-1200 Brussels, Belgium
Di Monaco, S:
Univ Turin, Dept Med Sci, Div Internal Med, Turin, Italy
Univ Turin, Dept Med Sci, Hypertens Unit, Turin, Italy
Warchol-Celinska, E:
Natl Inst Cardiol, Dept Hypertens, Warsaw, Poland
Lorthioir, A:
Hop Europeen Georges Pompidou, AP HP, Hypertens Unit, Paris, France
Hop Europeen Georges Pompidou, AP HP, DMU CARTE, Paris, France
Amar, L:
Hop Europeen Georges Pompidou, AP HP, Hypertens Unit, Paris, France
Hop Europeen Georges Pompidou, AP HP, DMU CARTE, Paris, France
Univ Paris, INSERM, CIC1418, Paris, France
Aparicio, LS:
Hosp Italiano Buenos Aires, Internal Med Dept, Hypertens Sect, Buenos Aires, DF, Argentina
Beauloye, C:
Catholic Univ Louvain, Div Cardiol, Clin Univ St Luc, 10 Ave Hippocrate, B-1200 Brussels, Belgium
Catholic Univ Louvain, Inst Rech Expt & Clin, Pole Cardiovasc Res, B-1200 Brussels, Belgium
Bruno, RM:
Univ Pisa, Dept Clin & Expt Med, Pisa, Italy
INSERM U970, Paris, France
Univ Paris, Paris, France
Chenu, P:
Catholic Univ Louvain, Div Cardiol, Clin Univ St Luc, 10 Ave Hippocrate, B-1200 Brussels, Belgium
Catholic Univ Louvain, Inst Rech Expt & Clin, Pole Cardiovasc Res, B-1200 Brussels, Belgium
de Leeuw, P:
Maastricht Univ, Dept Med, Med Ctr, Maastricht, Netherlands
De Backer, T:
Univ Hosp Ghent, Dept Cardiovasc Dis, Ghent, Belgium
Delmotte, P:
Ambroise Pare Univ Hosp, Hypertens Excellence Ctr, Dept Cardiol, Mons, Belgium
Dika, Z:
Univ Zagreb, Univ Hosp Ctr Zagreb, Sch Med, Dept Nephrol Arterial Hypertens Dialysis & Transp, Zagreb, Croatia
Gordin, D:
Univ Helsinki, Helsinki Univ Hosp, Helsinki Hypertens Ctr Excellence, Helsinki, Finland
Univ Helsinki, Helsinki Univ Hosp, Abdominal Ctr Nephrol, Helsinki, Finland
Biomedicum Helsinki, Folkhalsan Inst Genet, Folkhalsan Res Ctr, Helsinki, Finland
Heuten, H:
Univ Hosp Antwerp, Dept Cardiol, Edegem, Belgium
Iwashima, Y:
Dokkyo Med Univ, Dept Nephrol & Hypertens, Mibu, Tochigi, Japan
Krzesinski, JM:
Univ Liege Hosp, Dept Internal Med, Div Nephrol, Liege, Belgium
Univ Liege, Cardiovasc Sci, Grp Interdisciplinaire Genoproteom Appl GIGA, Liege, Belgium
Kroon, AA:
Maastricht Univ, Dept Med, Med Ctr, Maastricht, Netherlands
Mazzolai, L:
Lausanne Univ Hosp CHUV, Ctr Rare Vasc Dis, Heart & Vessel Dept, Div Angiol, Lausanne, Switzerland
RAVAD Registry, Lausanne, Switzerland
Poch, E:
Univ Barcelona, Hosp Clin Barcelona, Inst Invest Biomed August Pi i Sunyer IDIBAPS, Dept Nephrol & Kidney Transplantat, Barcelona, Spain
Sarafidis, P:
Aristotle Univ Thessaloniki, Hippokrat Hosp, Dept Nephrol, Thessaloniki, Greece
Seinturier, C:
Grenoble Alpes Univ Hosp, Competence Ctr Rare Vasc Dis, European Ctr Excellence Arterial Hypertens, Dept Cardiovasc Dis, Grenoble 9, France
Spiering, W:
Univ Utrecht, Univ Med Ctr Utrecht, Dept Vasc Med, Utrecht, Netherlands
Toubiana, L:
Sorbonne Univ, Univ Paris 13, INSERM, Sorbonne Paris Cite,UMR S1142,LIMICS,IRSAN, Paris, France
Van der Niepen, P:
Vrije Univ Brussel VUB, Univ Ziekenhuis Brussel, Dept Nephrol & Hypertens, Brussels, Belgium
van Twist, D:
Zuyderland Med Ctr, Dept Internal Med, Sittard Heerlen, Netherlands
Visona, A:
Azienda ULSS 2 Marca Trevigiana, Angiol Unit, Castelfranco Veneto, Italy
Wautrecht, JC:
Univ Hosp ULB Erasme, Dept Vasc Dis, Brussels, Belgium
Witowicz, H:
Natl Inst Cardiol, Dept Hypertens, Warsaw, Poland
Xu, JZ:
Shanghai Jiao Tong Univ, Ruijin Hosp, Shanghai Inst Hypertens, Dept Hypertens,Sch Med, Shanghai, Peoples R China
Prejbisz, A:
Natl Inst Cardiol, Dept Hypertens, Warsaw, Poland
Januszewicz, A:
Natl Inst Cardiol, Dept Hypertens, Warsaw, Poland
Azizi, M:
Hop Europeen Georges Pompidou, AP HP, Hypertens Unit, Paris, France
Hop Europeen Georges Pompidou, AP HP, DMU CARTE, Paris, France
Univ Paris, INSERM, CIC1418, Paris, France
Persu, A:
Catholic Univ Louvain, Div Cardiol, Clin Univ St Luc, 10 Ave Hippocrate, B-1200 Brussels, Belgium
Catholic Univ Louvain, Inst Rech Expt & Clin, Pole Cardiovasc Res, B-1200 Brussels, Belgium
De la Llama P.F.:
Hypertension Unit, Nephrology Department, Fundació Puigvert, Barcelona, Spain
Barros-Membrilla A.J.:
Cardiology Department, Hospital de la Santa Creu i Sant Pau, Barcelona, Spain
Green Published
|