Visceral Fibromuscular Dysplasia: from asymptomatic disorder to emergency.

Van der Niepen, Patricia;van Tussenbroek, Frank;Devos, Hannes;Debing, Erik;Persu, Alexandre;et.al.
(2018) European Journal of Clinical Investigation — Vol. 8, n° 11, p. e13023 (2018)

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Authors
  • Van der Niepen, Patricia
    Author
  • van Tussenbroek, Frank
    Author
  • Devos, Hannes
    Author
  • Debing, Erik
    Author
  • Di Monaco, SilviaUCLouvain
    Author
  • Goffette, PierreUCLouvain
    Author
  • Astarci, ParlaUCLouvain
    Author
  • Author
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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. This article is protected by copyright. All rights reserved.
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Citations

Van der Niepen, P., van Tussenbroek, F., Devos, H., Debing, E., Di Monaco, S., Goffette, P., Astarci, P., & Persu, A. (2018). Visceral Fibromuscular Dysplasia: from asymptomatic disorder to emergency. European Journal of Clinical Investigation, 8(11), e13023. https://doi.org/10.1111/eci.13023 (Original work published 2018)