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

Autor: 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, European/International FMD Registry and Initiative (FEIRI), and the Working Group ‘Hypertension and the Kidney’ of the European Society of Hypertension (ESH) Collaborators: Lucas, S Aparicio, Alexandre, Persu, Marco, Pappaccogli, Christophe, Beauloye, Patrick, Chenu, Frank, Hammer, Pierre, Goffette, Parla, Astarci, André, Peeters, Robert, Verhelst, Miikka, Vikkula, Patricia Van der Niepen, Frank Van Tussenbroek, Tine De Backer, Sofie, Gevaert, Dimitri, Hemelsoet, Luc, Defreyne, Hilde, Heuten, Laetitia, Yperzeele, Thijs Van der Zijden, Jean-Philippe, Lengelé, Jean-Marie, Krzesinski, Muriel, Sprynger, Philippe, Delmotte, Peter, Verhamme, Thomas, Vanassche, Pasquale, Scoppettuolo, Jean-Claude, Wautrecht, Wouter, Vinck, Vassilev, Dobrin, Yaneva, Teodora, Jiguang, Wang, Jianzhong, Xu, Bojan, Jelaković, Zivka, Dika, Daniel, Gordin, Ilkka, Tikkanen, Maarit, Venermo, N Mäkelä, R, Pierre-François, Plouin, Xavier, Jeunemaitre, Laurent, Toubiana, Michel, Azizi, Laurence, Amar, Antoine, Chédid, Elie, Mousseaux, Aurélien, Lorthioir, Olivier, Ormezzano, Christopher, Seinturier, Frédéric, Thony, Felix, Mahfoud, Saarraaken, Kulenthiran, Pantelis, Sarafidis, Alexia, Piperidou, Michael, Doumas, George, S Stergiou, Demetrios, Vlahakos, Caitriona, Canning, Yehonatan, Sharabi, Alberto, Morganti, Rosa Maria Bruno, Stefano, Taddei, Caterina, Romanini, Ilaria, Petrucci, Franco, Rabbia, Silvia Di Monaco, Gian Paolo Rossi, Silvia, Lerco, Minuz, Pietro, Mansueto, Giancarlo, DE MARCHI, Sergio, Marcon, Denise, Patrizia, Salice, Adriana, Visonà, Paola, Bigolin, Viviana, Zingaretti, Rosario, Cianci, Marialuisa, Zedde, Maria Chiara Matteucci, Yoshio, Iwashima, Osami, Kawarada, Yoshito, Kadoya, Daan, J van Twist, Bram, Kroon, Peter de Leeuw, Wilko, Spiering, Bert-Jan van den Born, Aud, Høieggen, Martin Skage Sommer, Andrzej, Januszewicz, Ewa, Warchoł-Celińska, Aleksander, Prejbisz, Adam, Witkowski, Helena, Witowicz, Jacek, Kądziela, Aleksandra, Soplińska, Krzysztof, Pieluszczak, Katarzyna, Jóżwik-Plebanek, Magdalena, Januszewicz, Elżbieta, Florczak, Piotr, Dobrowolski, Eva, Szabóová, Marek, Hudák, Matej, Moščovič, Juan Diego Mediavilla, Fernando Jaen Aguila, Anna, Oliveras, Julian, Segura, Jose, C Prado, Nicolas Roberto Robles, Esteban, Poch, Enrique, Montagud-Marrahi, Alicia, Molina, Elena, Guillen, Marta, Burrel, Patricia Fernàndez De la Llama, Antonio, J Barros-Membrilla, Anders, Gottsäter, Gregor, Wuerzner, Lucia, Mazzolai, Giacomo, Buso, Faiçal, Jarraya, Hanen, Chaker, David, Adlam, Constantina, Chrysochou, Neeraj, Dhaun, Robert, W Hunter, Iain, Macintyre, David, Webb
Přispěvatelé: Public and occupational health, Vascular Medicine, ACS - Atherosclerosis & ischemic syndromes, APH - Personalized Medicine, APH - Global Health, ACS - Heart failure & arrhythmias, Interne Geneeskunde, RS: MHeNs - R1 - Cognitive Neuropsychiatry and Clinical Neuroscience, RS: Carim - V02 Hypertension and target organ damage, MUMC+: MA Alg Interne Geneeskunde (9), Clinical sciences, Clinical Pharmacology and Clinical Pharmacy, Nephrology, CIC - HEGP (CIC 1418), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpital Européen Georges Pompidou [APHP] (HEGP), Assistance publique - Hôpitaux de Paris (AP-HP) (AP-HP)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Hôpitaux Universitaires Paris Ouest - Hôpitaux Universitaires Île de France Ouest (HUPO)-Institut National de la Santé et de la Recherche Médicale (INSERM)-Université Paris Cité (UPC), UCL - SSS/IREC/CARD - Pôle de recherche cardiovasculaire, UCL - (SLuc) Service de pathologie cardiovasculaire, UCL - SSS/IREC/IMAG - Pôle d'imagerie médicale, UCL - SSS/IONS/NEUR - Clinical Neuroscience, UCL - SSS/DDUV/GEHU - Génétique, UCL - (SLuc) Service de radiologie, UCL - (SLuc) Service de neurologie, UCL - (SLuc) Service de chirurgie cardiovasculaire et thoracique
Jazyk: angličtina
Rok vydání: 2021
Předmět:
Male
renovascular hypertension
Computed Tomography Angiography
Physiology
[SDV]Life Sciences [q-bio]
Fibromuscular dysplasia
030204 cardiovascular system & hematology
Magnetic resonance angiography
0302 clinical medicine
Risk Factors
Prevalence
Registries
Renovascular hypertension
Stroke
Computed tomography angiography
medicine.diagnostic_test
fibromuscular dysplasia
dissection
aneurysm
stroke
Incidence
Dissection
Age Factors
Middle Aged
Prognosis
3. Good health
Europe
Phenotype
Cohort
cardiovascular system
Female
Cardiology and Cardiovascular Medicine
circulatory and respiratory physiology
Adult
medicine.medical_specialty
Asia
Tunisia
Aneurysm
Argentina
Risk Assessment
03 medical and health sciences
Sex Factors
stomatognathic system
Predictive Value of Tests
Physiology (medical)
Internal medicine
medicine
Humans
cardiovascular diseases
Aged
business.industry
medicine.disease
Aortic Dissection
Stenosis
Angiography
business
Magnetic Resonance Angiography
030217 neurology & neurosurgery
Zdroj: Cardiovascular research, 117(3), 950-959. Oxford University Press
Cardiovascular Research, 117(3), 950-959. Oxford University Press
Cardiovascular Research
Cardiovascular Research, Oxford University Press (OUP), 2021, 117 (3), pp.950-959. ⟨10.1093/cvr/cvaa102⟩
Cardiovascular research, Vol. 117, no. 3, p. 950-959 (2021)
ISSN: 0008-6363
DOI: 10.1093/cvr/cvaa102⟩
Popis: 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 CTA, MRA and/or catheter-based angiography were eligible.Patients were predominantly women (82%) and Caucasians (88%). Age at diagnosis was 46±16 years (12% ≥65yo), 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 ≥65yo had more often multifocal FMD, lower eGFR 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. TRANSLATIONAL PERSPECTIVE: Fibromuscular dysplasia (FMD) is nowadays considered as a systemic arterial disease, warranting brain-to-pelvis vascular imaging in all patients. However, most current evidence is derived from a limited number of expert centres. Furthermore, one size may not fit all. Based on analysis of the first thousand patients enrolled in the European/International FMD registry (46 centres; 22 countries) we characterized distinct patient profiles according to FMD subtype, age and gender and identified predictors of widespread disease, aneurysms and dissections, paving the way for individualized management and follow-up. Further studies will allow refining patient characterization according to ethnicity, genetic profile and imaging biomarkers.
Databáze: OpenAIRE