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First International Consensus on the Diagnosis and Management Of VMJ0010.1177/1358863X18821816Vascular MedicineGornik, Persu et al. 821816research-article2019 Consensus Document Vascular Medicine 1 –26 First International Consensus on the diagnosis © The Author(s) 2019 Article reuse guidelines: and management of fibromuscular dysplasia sagepub.com/journals-permissions https://doi.org/10.1177/1358863X18821816DOI: 10.1177/1358863X18821816 journals.sagepub.com/home/vmj Heather L Gornik (Co-Chair)1*, Alexandre Persu (Co-Chair)2*, David Adlam3,4, Lucas S Aparicio5, Michel Azizi6,7,8, Marion Boulanger9, Rosa Maria Bruno10, Peter de Leeuw11, Natalia Fendrikova-Mahlay1, James Froehlich12, Santhi K Ganesh12, Bruce H Gray13, Cathlin Jamison14, Andrzej Januszewicz15, Xavier Jeunemaitre16,17, Daniella Kadian-Dodov18, Esther SH Kim19, Jason C Kovacic18, Pamela Mace20, Alberto Morganti21, Aditya Sharma22, Andrew M Southerland23, Emmanuel Touzé9, Patricia van der Niepen24, Jiguang Wang25, Ido Weinberg26, Scott Wilson27,28, Jeffrey W Olin18**, and Pierre-Francois Plouin6,7,8**, on behalf of the Working Group ‘Hypertension and the Kidney’ of the European Society of Hypertension (ESH) and the Society for Vascular Medicine (SVM) 1 Division of Cardiovascular Medicine, Department of Cardiovascular 19Division of Cardiovascular Medicine, Vanderbilt University Medical Medicine, University Hospitals Cleveland Medical Center and UH Center, Nashville, TN, USA Harrington Heart and Vascular Institute, Cleveland, OH, USA 20Fibromuscular Dysplasia Society of America (FMDSA), North Olmsted, 2Division of Cardiology, Department of Cardiovascular Diseases, OH, USA Cliniques Universitaires Saint-Luc and Pole of Cardiovascular Research, 21Centro Fisiologia Clinica e Ipertensione, Policlinico Hospital, Institut de Recherche Expérimentale et Clinique, Université Catholique University of Milan, Milan, Italy de Louvain, Brussels, Belgium 22Department of Medicine, Cardiovascular Medicine Division, University 3Department of Cardiovascular Sciences, Glenfield Hospital, Leicester, UK of Virginia, Charlottesville, VA, USA 4NIHR Leicester Biomedical Research Centre, Glenfield Hospital, 23Department of Neurology, University of Virginia, Charlottesville, Leicester, UK VA, USA 5Hypertension Section, Internal Medicine Department, Hospital Italiano 24Department of Nephrology & Hypertension Vrije Universiteit de Buenos Aires, Buenos Aires, Argentina Brussel (VUB), Universitair Ziekenhuis Brussel (UZ Brussel), 6Paris Descartes University, Paris, France Brussels, Belgium 7Assistance-Publique Hôpitaux de Paris, Hôpital Européen Georges 25Shanghai Institute of Hypertension and Center for Epidemiological Pompidou, Hypertension Unit, Paris, France Studies and Clinical Trials, Ruijin Hospital, Shanghai Jiaotong University 8Institut national de la santé et de la recherche médicale, Centre School of Medicine, Shanghai, China d’Investigation Clinique 1418, Paris, France 26Vascular Medicine Section and Vascular Center, Harvard Medical 9Normandie Université, UNICAEN, Inserm U1237, CHU Caen School, Massachusetts General Hospital, Boston, MA, USA Normandie, Caen, France 27Monash University (Central Clinical School of Medicine), Melbourne, 10Department of Clinical and Experimental Medicine University of Pisa, VIC, Australia Pisa, Italy 28Department of Renal Medicine, Alfred Health, Melbourne, VIC, 11Department of Medicine, Maastricht University Medical Center, Australia Maastricht, The Netherlands 12Department of Internal Medicine, Frankel Cardiovascular Center, *These authors contributed equally to this work. University of Michigan, Ann Arbor, MI, USA **These authors contributed equally to this work. 13University of South Carolina School of Medicine/Greenville, This contribution is being co-published in the following journals: Journal of Greenville, SC, USA Hypertension and Vascular Medicine. There will be minor differences in the 14Association belge de patients atteints de Dysplasie Fibromusculaire/ version published in Vascular Medicine due to copy-editing differences. FMD Groep België (FMD-Be), Brussels, Belgium 15Department of Hypertension, Institute of Cardiology, Warsaw, Poland 16APHP, Department of Genetics and Centre for Rare Vascular Corresponding author: Diseases, Hôpital Européen Georges Pompidou, Paris, France Heather L Gornik, Division of Cardiovascular Medicine, Department of 17INSERM, U970 – PARCC, University Paris Descartes, Sorbonne Paris Cardiovascular Medicine, University Hospitals Cleveland Medical Center Cité, Paris, France and UH Harrington Heart and Vascular Institute, 11000 Euclid Avenue, 18Zena and Michael A Wiener Cardiovascular Institute and Marie-Josée Cleveland, Ohio, 44106, USA. and Henry R. Kravis Center for Cardiovascular Health; Icahn School of Email: [email protected] Medicine at Mount Sinai, New York, NY, USA Twitter: @heatherlgornik 2 Vascular Medicine 00(0) Abstract 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. Keywords aneurysm, cervical artery dissection, fibromuscular dysplasia (FMD), percutaneous angioplasty, pressure gradients, renovascular hypertension, spontaneous coronary artery dissection Introduction field, including data from observational registries of patients with FMD, the authors acknowledge that level I data in this field Fibromuscular dysplasia (FMD) is a non-atherosclerotic are limited and the majority of points are based upon the expert arterial disease that is characterized by abnormal cellular consensus of the international panel of writing committee mem- proliferation and distorted architecture of the arterial wall. bers. It is the intent of the writing committee that this interna- FMD primarily manifests as beaded (multifocal) or focal tional consensus document, including identification of research lesions in medium or small-sized arteries, though the clini- priorities, will lead to future high-quality research efforts, addi- cal phenotype of FMD has recently been expanded to tional observational studies, and randomized controlled trials, 1,2 include arterial dissection, aneurysm, and tortuosity. and that these data will be incorporated into a future interna- FMD most commonly affects the renal and extracranial tional guideline document. carotid and vertebral arteries, but nearly all arterial beds Although the writing committee recognizes the impor- may be affected, and multivessel involvement is common. tance of FMD as a cause of renovascular hypertension in 2,3 Approximately 80–90% of patients with FMD are women. children, the scope of this document is focused on FMD in Though less common, men also develop FMD and may adult patients. Writing committee members were selected have a more aggressive course with a higher frequency of by each society based upon extensive experience in the 3 aneurysms and dissections. Though initially described in care of patients with FMD and/or research contributions to 1938 and classified according to angiographic and histo- the field, including participation in international FMD reg- pathological findings in the 1960s and 1970s, the greatest istries. This document has been peer reviewed by members advances in the understanding of the pathophysiology and of both the ESH and SVM, and this final expert consensus natural history of FMD have come in the past decade and has been endorsed by both the working group ‘Hypertension have been driven by data from international patient regis- and the Kidney’ of the ESH and the Board of Trustees of 2,4 tries and multicenter research collaborations. In 2014, the SVM. multispecialty groups from Europe and the United States published consensus statements regarding FMD.5,6 Although these documents were developed independently, Definition, classification, and there were many similarities in interpretation of the medi- differential diagnosis cal literature and state of the clinical science – both docu- ments representing initial attempts to develop a Definition of FMD multispecialty consensus on a standardized approach to this The European consensus definition of FMD provides a disease. baseline description of what constitutes FMD: An idio- Building upon the prior European and US documents, as pathic, segmental, non-atherosclerotic and non-inflamma- well as international symposia held in Cleveland, Ohio, USA tory disease of the musculature of arterial walls, leading to (May 18–19, 2017) and Brussels, Belgium (February 22–24, stenosis of small and medium-sized arteries.6 Lesions of 2018), a writing committee was commissioned by the Society FMD can be either symptomatic or clinically silent and can for Vascular Medicine (SVM) and the working group be either hemodynamically significant or not. The diagnosis ‘Hypertension and the Kidney’ of the European Society for of FMD
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