The European/International Fibromuscular Dysplasia Registry and Initiative (FEIRI)—clinical phenotypes and their predictors based on a cohort of 1000 patients
Authors: Pappaccogli Marco , Di Monaco Silvia , Warchoł-Celińska Ewa , Lorthioir Aurélien , Amar Laurence , Aparicio Lucas S , Beauloye Christophe , Bruno Rosa Maria , Chenu Patrick , de Leeuw Peter , De Backer Tine , Delmotte Philippe , Dika Zivka , Gordin Daniel , Heuten Hilde , Iwashima Yoshio , Krzesinski Jean-Marie , Kroon Abraham A , Mazzolai Lucia , Poch Esteban , Sarafidis Pantelis , Seinturier Christophe , Spiering Wilko , Toubiana Laurent , Van der Niepen Patricia , van Twist Daan , Visonà Adriana , Wautrecht Jean-Claude , Witowicz Helena , Xu Jianzhong , Prejbisz Aleksander , Januszewicz Andrzej , Azizi Michel , Persu Alexandre ,   the European/International FMD Registry and Initiative (FEIRI), and the Working Group ‘Hypertension and the Kidney’ of the European Society of Hypertension (ESH), 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 
Published: April 2020

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.