Visceral Fibromuscular Dysplasia: From asymptomatic disorder to emergency.

Patricia Van der Niepen, Frank van Tussenbroek, Hannes Devos, Erik Debing, Silvia Di Monaco, Pierre Goffette, Parla Astarci, Alexandre Persu

Journal: European journal of clinical investigation 2019;48(11):e13023

PMID: 30156710

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Abstract

Fibromuscular dysplasia (FMD) is an idiopathic, segmental, non-atherosclerotic and non-inflammatory disease of the musculature of arterial walls, leading to stenosis of small and medium-sized arteries, mostly involving renal and cervical arteries. As a result of better and more systematic screening, it appears that involvement of the splanchnic vascular bed is more frequent than originally assumed. We review epidemiology, pathogenesis, clinical picture as well as diagnosis and treatment of visceral artery (VA) FMD. The clinical picture is very diverse, and diagnosis is based on CT-, MR- or conventional catheter-based angiography. Involvement of VAs generally occurs among patients with multi-vessel FMD. Therefore, screening for VA FMD is advised especially in renal artery (RA) FMD and in case of aneurysms and/or dissections. Treatment depends on the clinical picture. However, the level of evidence is low, and much of the common practice is extrapolated from visceral atherosclerotic disease.

Address: Department of Nephrology & Hypertension, Universitair Ziekenhuis Brussel. Vrije Universiteit Brussel, Brussels, Belgium.; Department of Radiology, Universitair Ziekenhuis Brussel. Vrije Universiteit Brussel, Brussels, Belgium.; Department of Vascular Surgery, Universitair Ziekenhuis Brussel. Vrije Universiteit Brussel, Brussels, Belgium.; Division of Cardiology, Cliniques Universitaires Saint-Luc, Université Catholique de Louvain, Brussels, Belgium.; Internal Medicine and Hypertension Division, Department of Medical Sciences, AOU Città della Salute e della Scienza, University of Turin, Turin, Italy.; Department of Radiology, Cliniques Universitaires Saint-Luc, Université Catholique de Louvain, Brussels, Belgium.; Division of Vascular Surgery, Cliniques Universitaires Saint-Luc, Université Catholique de Louvain, Brussels, Belgium.; Pole of Cardiovascular Research, Institut de Recherche Expérimentale et Clinique, Université Catholique de Louvain, Brussels, Belgium.; Division of Cardiology, Cliniques Universitaires Saint-Luc, Université Catholique de Louvain, Brussels, Belgium.; Pole of Cardiovascular Research, Institut de Recherche Expérimentale et Clinique, Université Catholique de Louvain, Brussels, Belgium.

DOI: 10.1111/eci.13023

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MeSH Terms: Aged, Aged, 80 and over, Angiography, Asymptomatic Diseases, Early Diagnosis, Emergencies, Emergency Treatment, Female, Fibromuscular Dysplasia, Healthy Lifestyle, Humans, Male, Platelet Aggregation Inhibitors, Reperfusion, Viscera