First International Consensus on the diagnosis and management of fibromuscular dysplasia

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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.

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Clinical features, management, and prognosis of spontaneous coronary artery dissection.

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Spontaneous coronary artery dissection (SCAD) is an acute coronary event of uncertain origin. Clinical features and prognosis remain insufficiently characterized.

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Nonatherosclerotic spontaneous coronary artery dissection (NA-SCAD) is underdiagnosed and an important cause of myocardial infarction in young women. The frequency of predisposing and precipitating conditions and cardiovascular outcomes remains poorly described.

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Neurofibromatosis type 1

David Gutmann, Rosalie E Ferner, Robert H. Listernick … (2017)

Neurofibromatosis type 1 is a complex autosomal dominant disorder caused by germline mutations in the NF1 tumour suppressor gene. Nearly all individuals with neurofibromatosis type 1 develop pigmentary lesions (café-au-lait macules, skinfold freckling and Lisch nodules) and dermal neurofibromas. Some individuals develop skeletal abnormalities (scoliosis, tibial pseudarthrosis and orbital dysplasia), brain tumours (optic pathway gliomas and glioblastoma), peripheral nerve tumours (spinal neurofibromas, plexiform neurofibromas and malignant peripheral nerve sheath tumours), learning disabilities, attention deficits, and social and behavioural problems, which can negatively affect quality of life. With the identification of NF1 and the generation of accurate preclinical mouse strains that model some of these clinical features, therapies that target the underlying molecular and cellular pathophysiology for neurofibromatosis type 1 are becoming available. Although no single treatment exists, current clinical management strategies include early detection of disease phenotypes (risk assessment) and biologically targeted therapies. Similarly, new medical and behavioural interventions are emerging to improve the quality of life of patients. Although considerable progress has been made in understanding this condition, numerous challenges remain; a collaborative and interdisciplinary approach is required to manage individuals with neurofibromatosis type1 and to develop effective treatments.

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Author and article information

Journal

Title: Vascular Medicine

Abbreviated Title: Vasc Med

Publisher: SAGE Publications

ISSN (Print): 1358-863X

ISSN (Electronic): 1477-0377

Publication date Created: January 16 2019

Publication date (Electronic): January 16 2019

Page: 1358863X1882181

Affiliations

[1 ]Division of Cardiovascular Medicine, Department of Cardiovascular Medicine, University Hospitals Cleveland Medical Center and UH Harrington Heart and Vascular Institute, Cleveland, OH, USA

[2 ]Division 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

[3 ]Department of Cardiovascular Sciences, Glenfield Hospital, Leicester, UK

[4 ]NIHR Leicester Biomedical Research Centre, Glenfield Hospital, Leicester, UK

[5 ]Hypertension Section, Internal Medicine Department, Hospital Italiano de Buenos Aires, Buenos Aires, Argentina

[6 ]Paris Descartes University, Paris, France

[7 ]Assistance-Publique Hôpitaux de Paris, Hôpital Européen Georges Pompidou, Hypertension Unit, Paris, France

[8 ]Institut national de la santé et de la recherche médicale, Centre d’Investigation Clinique 1418, Paris, France

[9 ]Normandie Université, UNICAEN, Inserm U1237, CHU Caen Normandie, Caen, France

[10 ]Department of Clinical and Experimental Medicine University of Pisa, Pisa, Italy

[11 ]Department of Medicine, Maastricht University Medical Center, Maastricht, The Netherlands

[12 ]Department of Internal Medicine, Frankel Cardiovascular Center, University of Michigan, Ann Arbor, MI, USA

[13 ]University of South Carolina School of Medicine/Greenville, Greenville, SC, USA

[14 ]Association belge de patients atteints de Dysplasie Fibromusculaire/FMD Groep België (FMD-Be), Brussels, Belgium

[15 ]Department of Hypertension, Institute of Cardiology, Warsaw, Poland

[16 ]APHP, Department of Genetics and Centre for Rare Vascular Diseases, Hôpital Européen Georges Pompidou, Paris, France

[17 ]INSERM, U970 – PARCC, University Paris Descartes, Sorbonne Paris Cité, Paris, France

[18 ]Zena 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, NY, USA

[19 ]Division of Cardiovascular Medicine, Vanderbilt University Medical Center, Nashville, TN, USA

[20 ]Fibromuscular Dysplasia Society of America (FMDSA), North Olmsted, OH, USA

[21 ]Centro Fisiologia Clinica e Ipertensione, Policlinico Hospital, University of Milan, Milan, Italy

[22 ]Department of Medicine, Cardiovascular Medicine Division, University of Virginia, Charlottesville, VA, USA

[23 ]Department of Neurology, University of Virginia, Charlottesville, VA, USA

[24 ]Department of Nephrology & Hypertension Vrije Universiteit Brussel (VUB), Universitair Ziekenhuis Brussel (UZ Brussel), Brussels, Belgium

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

[26 ]Vascular Medicine Section and Vascular Center, Harvard Medical School, Massachusetts General Hospital, Boston, MA, USA

[27 ]Monash University (Central Clinical School of Medicine), Melbourne, VIC, Australia

[28 ]Department of Renal Medicine, Alfred Health, Melbourne, VIC, Australia

Article

DOI: 10.1177/1358863X18821816

PubMed ID: 30648921

SO-VID: 1dedc07b-cb78-4ccb-9c64-39a6138c6908

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http://journals.sagepub.com/page/policies/text-and-data-mining-license

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First International Consensus on the diagnosis and management of fibromuscular dysplasia

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International Journal of Management Studies

Most cited references 154

Clinical features, management, and prognosis of spontaneous coronary artery dissection.

Spontaneous coronary artery dissection: association with predisposing arteriopathies and precipitating stressors and cardiovascular outcomes.

Neurofibromatosis type 1

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