Secondary Logo

Institutional members access full text with Ovid®

Share this article on:

First international consensus on the diagnosis and management of fibromuscular dysplasia

Gornik, Heather L.a,*; Persu, Alexandreb,*; Adlam, Davidc,d; Aparicio, Lucas S.e; Azizi, Michelf,g,h; Boulanger, Marioni; Bruno, Rosa M.j; De Leeuw, Peterk; Fendrikova-Mahlay, Nataliaa; Froehlich, Jamesl; Ganesh, Santhi K.l; Gray, Bruce H.m; Jamison, Cathlinn; Januszewicz, Andrzejo; Jeunemaitre, Xavierp,q; Kadian-Dodov, Daniellar; Kim, Esther S.H.s; Kovacic, Jason C.r; Mace, Pamelat; Morganti, Albertou; Sharma, Adityav; Southerland, Andrew M.w; Touzé, Emmanueli; Van der Niepen, Patriciax; Wang, Jiguangy; Weinberg, Idoz; Wilson, Scottaa,bb; Olin, Jeffrey W.r,†; Plouin, Pierre-Francoisf,g,h,† on behalf of the Working Group ‘Hypertension and the Kidney’ of the European Society of Hypertension (ESH) and the Society for Vascular Medicine (SVM)

doi: 10.1097/HJH.0000000000002019

This article is a comprehensive document on the diagnosis and management of fibromuscular dysplasia (FMD) which was commissioned by the Working Group ‘Hypertension and the Kidney’ of the European Society of Hypertension (ESH) and the Society for Vascular Medicine (SVM). This document updates previous consensus documents/scientific statements on FMD published in 2014 with full harmonization of the position of European and US experts. In addition to practical consensus-based clinical recommendations, including a consensus protocol for catheter-based angiography and percutaneous angioplasty for renal FMD, the document also includes the first analysis of the European/International FMD Registry and provides updated data from the US Registry for FMD. Finally, it provides insights on ongoing research programs and proposes future research directions for understanding this multifaceted arterial disease.

aDivision of Cardiovascular Medicine, Department of Cardiovascular Medicine, University Hospitals Cleveland Medical Center and UH Harrington Heart and Vascular Institute, Cleveland, Ohio, USA

bDivision of Cardiology, Department of Cardiovascular Diseases, Cliniques Universitaires Saint-Luc and Pole of Cardiovascular Research, Institut de Recherche Expérimentale et Clinique, Université Catholique de Louvain, Brussels, Belgium

cDepartment of Cardiovascular Sciences

dNIHR Leicester Biomedical Research Centre, Glenfield Hospital, Leicester, UK

eHypertension Section, Internal Medicine Department, Hospital Italiano de Buenos Aires, Buenos Aires, Argentina

fParis Descartes University

gHypertension Unit, Assistance-Publique Hôpitaux de Paris, Hôpital Européen Georges Pompidou

hInstitut National de la Santé et de la Recherche Médicale, Centre d’Investigation Clinique 1418, Paris

iNormandie Université, UNICAEN, Inserm U1237, CHU Caen Normandie, Caen, France

jDepartment of Clinical and Experimental Medicine, University of Pisa, Pisa, Italy

kDepartment of Medicine, Maastricht University Medical Center, Maastricht, The Netherlands

lFrankel Cardiovascular Center, Department of Internal Medicine, University of Michigan, Ann Arbor, Michigan

mUniversity of South Carolina School of Medicine Greenville, Greenville, South Carolina, USA

nAssociation Belge De Patients Atteints De Dysplasie Fibromusculaire/FMD Groep België (FMD-Be), Brussels, Belgium

oDepartment of Hypertension, Institute of Cardiology, Warsaw, Poland

pAPHP, Department of Genetics and Centre for Rare Vascular Diseases, Hôpital Européen Georges Pompidou

qINSERM, U970 – PARCC, University Paris Descartes, Sorbonne Paris Cité, Paris, France

rZena and Michael A. Wiener Cardiovascular Institute and Marie-Josée and Henry R. Kravis Center for Cardiovascular Health, Icahn School of Medicine at Mount Sinai, New York

sDivision of Cardiovascular Medicine, Vanderbilt University Medical Center, Nashville, Tennessee

tFibromuscular Dysplasia Society of America (FMDSA), North Olmsted, Ohio, USA

uCentro Fisiologia Clinica e Ipertensione, Policlinico Hospital, University of Milan, Milan, Italy

vCardiovascular Medicine Division, Department of Medicine

wDepartment of Neurology, University of Virginia, Charlottesville, Virginia, USA

xDepartment of Nephrology and Hypertension, Vrije Universiteit Brussel (VUB), Universitair Ziekenhuis Brussel (UZ Brussel), Brussels, Belgium

yShanghai Institute of Hypertension and Center for Epidemiological Studies and Clinical Trials, Ruijin Hospital, Shanghai Jiaotong University School of Medicine, Shanghai, China

zVascular Medicine Section and Vascular Center, Harvard Medical School, Massachusetts General Hospital, Boston, Massachusetts, USA

aaMonash University (Central Clinical School of Medicine)

bbDepartment of Renal Medicine, Alfred Health, Melbourne, Victoria, Australia

Correspondence to Alexandre Persu, Division of Cardiology, Department of Cardiovascular Diseases, Cliniques Universitaires Saint Luc (UCL), 10 Avenue Hippocrate, 1200 Brussels, Belgium. Tel: +32 2 764 63 06; fax: +32 2 764 28 36; e-mail:

Abbreviations: AHA, American Heart Association; AMI, acute myocardial infarction; ARB, AT1 blockers; ARCADIA study, Assessment of Renal and Cervical Artery Dysplasia study; ARCADIA-POL study, Assessment of Renal and Cervical Artery Dysplasia–Poland study; CeAD, cervical artery dissection; CTA, computed tomographic angiography; ESH, European Society of Hypertension; FMD, fibromuscular dysplasia; ICA, internal carotid artery; MRA, magnetic resonance angiography; OCT, optical coherence tomography; ROKD-FMD, (Australian) Registry of Kidney Disease-Fibromuscular Dysplasia; SCAD, spontaneous coronary artery dissection; SNP, single nucleotide polymorphism; SVM, Society for Vascular Medicine; TGF-beta1, transforming growth factor-beta 1; TGF-beta2, transforming growth factor-beta 2; TIA, transient ischemic attack

Received 4 April, 2018

Accepted 9 November, 2018

This contribution is being co-published in the following journals: Journal of Hypertension and Vascular Medicine.

Supplemental digital content is available for this article. Direct URL citations appear in the printed text and are provided in the HTML and PDF versions of this article on the journal's Website (

Copyright © 2019 Wolters Kluwer Health, Inc. All rights reserved.