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Fibromuscular dysplasia and hypertension—a statement on behalf of the British and Irish Hypertension Society

Journal of Human Hypertension volume 35, pages 1051–1053 (2021)Cite this article

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Fibromuscular dysplasia (FMD) is an idiopathic, non-atherosclerotic and non-inflammatory disease of the arterial wall usually involving medium or small-sized arteries [1]. FMD most commonly affects the renal and extracranial carotid and vertebral arteries, but nearly all arterial beds may be involved [1]. Although originally described as a rare disease [2], particularly affecting young women, its prevalence is now estimated to be 3–4% of the general population and even higher in subjects with hypertension [3, 4]. FMD together with other genetic conditions like Neurofibromatosis 1, Williams syndrome and Alagille syndrome may account for up to 10% of cases of secondary hypertension in children and young people [5].

Clinical manifestations of FMD depend on the vascular beds affected. In children, as opposed to adults, FMD is more common in boys with occasional reports of associated mid aortic syndrome. In adults, involvement of the renal arteries is often associated with hypertension and more than 85% of adults included in The European/International Fibromuscular Dysplasia Registry and Initiative (FEIRI) have pre-hypertension or hypertension [6]. Thus, hypertension specialists should play a key role in the diagnosis and management of the condition, but so far no focused guidance is available in the UK. With the widespread use of non-invasive imaging techniques of the vasculature, more adult patients without the classical features of renal FMD are being diagnosed. This supports that the view that FMD is less rare than initially thought but raises new questions regarding the diagnostic workup for the exclusion of secondary causes of hypertension given that our knowledge is mostly derived from the analysis of symptomatic cases in international registries [1].

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References

  1. Gornik HL, Persu A, Adlam D, Aparicio LS, Azizi M, Boulanger M, et al. First International Consensus on the diagnosis and management of fibromuscular dysplasia. Vasc Med. 2019;24:164–89.

    Article  Google Scholar 

  2. Plouin PF, Perdu J, La Batide-Alanore A, Boutouyrie P, Gimenez-Roqueplo AP, Jeunemaitre X. Fibromuscular dysplasia. Orphanet J Rare Dis. 2007;2:1–8.

    Article  Google Scholar 

  3. Shivapour DM, Erwin P, Kim ESH. Epidemiology of fibromuscular dysplasia: a review of the literature. Vasc Med. 2016;21:376–81.

    Article  Google Scholar 

  4. Hendricks NJ, Matsumoto AH, Angle JF, Baheti A, Sabri SS, Park AW, et al. Is fibromuscular dysplasia underdiagnosed? A comparison of the prevalence of FMD seen in CORAL trial participants versus a single institution population of renal donor candidates. Vasc Med. 2014;19:363–7.

    Article  Google Scholar 

  5. Tullus K. Renovascular hypertension in children. Pediatr Hypertens. 2013;371:367–77.

    Article  Google Scholar 

  6. Pappaccogli M, Di Monaco S, Warchoł-Celińska E, Lorthioir A, Amar L, Aparicio L, et al. The European/International Fibromuscular Dysplasia Registry and Initiative (FEIRI)—clinical phenotypes and their predictors based on a cohort of 1000 patients. Cardiovasc Res. 2020;cvaa102. [Epub ahead of print].

  7. Dobrowolski P, Januszewicz M, Witowicz H, Warchoł-Celińska E, Klisiewicz A, Skrzypczyńska-Banasik U, et al. Prevalence of smoking and clinical characteristics in fibromuscular dysplasia. The ARCADIA-POL study. Blood Press. 2019;28:49–56.

    Article  Google Scholar 

  8. Januszewicz M, Januszewicz A, Michałowska I, Klisiewicz A, Dobrowolski P, Warchoł-Celińska E, et al. Association of intrarenal blood flow with renal function and target organ damage in hypertensive patients with fibromuscular dysplasia: The ARCADIA-POL study. Pol Arch Intern Med. 2019;129:234–41.

    PubMed  Google Scholar 

  9. National Institute for Health and Care Excellence [NICE]. Hypertension in adults: diagnosis and management (NICE Guideline CG136). 2019. https://www.nice.org.uk/guidance/ng136.

  10. Morselli F, Karunanithy N, Chowienczyk PJ, Faconti L. Do we need more vascular imaging for the screening of secondary hypertension? Mid-aortic syndrome in a young male adult. J Hum Hypertens. 2020;34:668–70.

    Article  CAS  Google Scholar 

  11. Beregi J, Lovegny S, Gautier C, Mounier-Vehier C, Moretti A, Desmouchelle F, et al. Fibromuscular dysplasia of the renal arteries: comparison of helical CT angiography and arteriography. Am J Roentgenol. 1999;172:27–34.

    Article  CAS  Google Scholar 

  12. Willoteaux S, Faivre-Pierret M, Moranne O, Lions C, Bruzzi J, Finot M, et al. Fibromuscular dysplasia of the main renal arteries: comparison of contrast-enhanced MR angiography with digital subtraction angiography. Radiology. 2006;241:922–9.

    Article  Google Scholar 

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British and Irish Hypertension Society

Luca Faconti1, Franca Morselli1, Manish Sinha2, Constantina Chrysochou3, Philip J. Chowienczyk1

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  1. Department of Clinical Pharmacology, King’s College London British Heart Foundation Centre, London, UK

    Luca Faconti, Franca Morselli & Philip J. Chowienczyk

  2. Department of Paediatric Nephrology, Evelina London Children’s Hospital, London, UK

    Manish Sinha

  3. Renal Department, Salford Royal NHS Foundation Trust, Salford, UK

    Constantina Chrysochou

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British and Irish Hypertension Society

  • Luca Faconti
  • , Franca Morselli
  • , Manish Sinha
  • , Constantina Chrysochou
  •  & Philip J. Chowienczyk

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Correspondence to Luca Faconti.

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Faconti, L., Morselli, F., Sinha, M. et al. Fibromuscular dysplasia and hypertension—a statement on behalf of the British and Irish Hypertension Society. J Hum Hypertens 35, 1051–1053 (2021). https://doi.org/10.1038/s41371-020-00456-6

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