The European/International Fibromuscular Dysplasia Registry and Initiative (FEIRI)—clinical phenotypes and their predictors based on a cohort of 1000 patients
Author:
Pappaccogli Marco123ORCID, Di Monaco Silvia1ORCID, Warchoł-Celińska Ewa4, Lorthioir Aurélien5ORCID, Amar Laurence56, Aparicio Lucas S7ORCID, Beauloye Christophe23, Bruno Rosa Maria89ORCID, Chenu Patrick23, de Leeuw Peter10, De Backer Tine11, Delmotte Philippe12, Dika Zivka13ORCID, Gordin Daniel141516ORCID, Heuten Hilde17, Iwashima Yoshio18, Krzesinski Jean-Marie1920ORCID, Kroon Abraham A10ORCID, Mazzolai Lucia21, Poch Esteban22ORCID, Sarafidis Pantelis23ORCID, Seinturier Christophe24, Spiering Wilko25, Toubiana Laurent26, Van der Niepen Patricia27ORCID, van Twist Daan28, Visonà Adriana2930, Wautrecht Jean-Claude31ORCID, Witowicz Helena4, Xu Jianzhong32, Prejbisz Aleksander4ORCID, Januszewicz Andrzej4, Azizi Michel56, Persu Alexandre23ORCID, Aparicio Lucas S, Persu Alexandre, Pappaccogli Marco, Beauloye Christophe, Chenu Patrick, Hammer Frank, Goffette Pierre, Astarci Parla, Peeters André, Verhelst Robert, Vikkula Miikka, Van der Niepen Patricia, Van Tussenbroek Frank, De Backer Tine, Gevaert Sofie, Hemelsoet Dimitri, Defreyne Luc, Heuten Hilde, Yperzeele Laetitia, Van der Zijden Thijs, Lengelé Jean-Philippe, Krzesinski Jean-Marie, Sprynger Muriel, Delmotte Philippe, Verhamme Peter, Vanassche Thomas, Scoppettuolo Pasquale, Wautrecht Jean-Claude, Vinck Wouter, Dobrin Vassilev, Teodora Yaneva, Wang Jiguang, Xu Jianzhong, Jelaković Bojan, Dika Zivka, Gordin Daniel, Tikkanen Ilkka, Venermo Maarit, Mäkelä R N, Plouin Pierre-François, Jeunemaitre Xavier, Toubiana Laurent, Azizi Michel, Amar Laurence, Chédid Antoine, Mousseaux Elie, Lorthioir Aurélien, Ormezzano Olivier, Seinturier Christopher, Thony Frédéric, Mahfoud Felix, Kulenthiran Saarraaken, Sarafidis Pantelis, Piperidou Alexia, Doumas Michael, Stergiou George S, Vlahakos Demetrios, Canning Caitriona, Sharabi Yehonatan, Morganti Alberto, Bruno Rosa Maria, Taddei Stefano, Romanini Caterina, Petrucci Ilaria, Rabbia Franco, Pappaccogli Marco, Di Monaco Silvia, Rossi Gian Paolo, Lerco Silvia, Minuz Pietro, Mansueto Giancarlo, De Marchi Sergio, Marcon Denise, Salice Patrizia, Visonà Adriana, Bigolin Paola, Zingaretti Viviana, Cianci Rosario, Zedde Marialuisa, Matteucci Maria Chiara, Iwashima Yoshio, Kawarada Osami, Kadoya Yoshito, van Twist Daan J, Kroon Bram, de Leeuw Peter, Spiering Wilko, van den Born Bert-Jan, Høieggen Aud, Sommer Martin Skage, Januszewicz Andrzej, Warchoł-Celińska Ewa, Prejbisz Aleksander, Witkowski Adam, Witowicz Helena, Kądziela Jacek, Soplińska Aleksandra, Pieluszczak Krzysztof, Jóżwik-Plebanek Katarzyna, Januszewicz Magdalena, Florczak Elżbieta, Dobrowolski Piotr, Szabóová Eva, Hudák Marek, Moščovič Matej, Mediavilla Juan Diego, Aguila Fernando Jaen, Oliveras Anna, Segura Julian, Prado Jose C, Robles Nicolas Roberto, Poch Esteban, Montagud-Marrahi Enrique, Molina Alicia, Guillen Elena, Burrel Marta, De la Llama Patricia Fernàndez, Barros-Membrilla Antonio J, Gottsäter Anders, Wuerzner Gregor, Mazzolai Lucia, Buso Giacomo, Jarraya Faiçal, Chaker Hanen, Adlam David, Chrysochou Constantina, Dhaun Neeraj, Hunter Robert W, MacIntyre Iain, Webb David,
Affiliation:
1. Division of Internal Medicine and Hypertension Unit, Department of Medical Sciences, University of Turin, Turin, Italy 2. Division of Cardiology, Cliniques Universitaires Saint-Luc, Université Catholique de Louvain, 10 Avenue Hippocrate, 1200 Brussels, Belgium 3. Pole of Cardiovascular Research, Institut de Recherche Expérimentale et Clinique, Université Catholique de Louvain, 1200 Brussels, Belgium 4. Department of Hypertension, National Institute of Cardiology, Warsaw, Poland 5. Hypertension Unit and DMU CARTE, AP-HP, Hôpital Européen Georges Pompidou, Paris, France 6. Université de Paris, CIC1418, INSERM, Paris, France 7. Hypertension Section, Internal Medicine Department, Hospital Italiano de Buenos Aires, Buenos Aires, Argentina 8. Department of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy 9. INSERM U970 and Université de Paris, Paris, France 10. Department of Medicine, Maastricht University Medical Center, Maastricht, The Netherlands 11. Department of Cardiovascular Diseases, University Hospital Ghent, Ghent, Belgium 12. Hypertension Excellence Centre, Department of Cardiology, Ambroise Paré University Hospital, Mons, Belgium 13. Department of Nephrology, Arterial Hypertension, Dialysis and Transplantation, University Hospital Center Zagreb, School of Medicine University of Zagreb, Zagreb, Croatia 14. Helsinki Hypertension Centre of Excellence, University of Helsinki, Helsinki University Hospital, Helsinki, Finland 15. Abdominal Center Nephrology, University of Helsinki, Helsinki University Hospital, Helsinki, Finland 16. Folkhälsan Institute of Genetics, Folkhälsan Research Centre, Biomedicum Helsinki, Helsinki, Finland 17. Department of Cardiology, University Hospital of Antwerp, Edegem, Belgium 18. Department of Nephrology and Hypertension, Dokkyo Medical University, Tochigi, Japan 19. Division of Nephrology, Department of Internal Medicine, University of Liège Hospital, Liège, Belgium 20. Groupe Interdisciplinaire de Génoprotéomique Appliquée (GIGA), Cardiovascular Sciences, University of Liège, Liège, Belgium 21. Division of Angiology, Heart and Vessel Department, Lausanne University Hospital—CHUV, Centre of Rare Vascular Diseases and RAVAD Registry, Lausanne, Switzerland 22. Department of Nephrology and Kidney Transplantation, Hospital Clínic of Barcelona, Institut D’Investigacions Biomèdiques August Pi i Sunyer (IDIBAPS), University of Barcelona, Barcelona, Spain 23. Department of Nephrology, Hippokration Hospital, Aristotle University of Thessaloniki, Thessaloniki, Greece 24. Department of Cardiovascular Disease, Competence Center for Rare Vascular Diseases, European Center of Excellence in Arterial Hypertension, Grenoble-Alpes University Hospital, CS Grenoble Cedex 9, France 25. Department of Vascular Medicine, University Medical Center Utrecht, Utrecht University, Utrecht, The Netherlands 26. Sorbonne Université, Université Paris 13, Sorbonne Paris Cité, INSERM, UMR_S1142, LIMICS, IRSAN, Paris, France 27. Department of Nephrology & Hypertension, Universitair Ziekenhuis Brussel , Vrije Universiteit Brussel (VUB), Brussels, Belgium 28. Department of Internal Medicine, Zuyderland Medical Centre, Sittard/Heerlen, The Netherlands 29. Angiology Unit, Azienda ULSS 2 Marca Trevigiana, Castelfranco Veneto, Italy 30. ESVM Board Member 31. Department of Vascular Diseases, University Hospital ULB Erasme, Brussels, Belgium 32. Shanghai Institute of Hypertension, Department of Hypertension, Ruijin Hospital, Shanghai Jiaotong University School of Medicine, Shanghai, China
Abstract
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.
Publisher
Oxford University Press (OUP)
Subject
Physiology (medical),Cardiology and Cardiovascular Medicine,Physiology
Cited by
36 articles.
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