The arterial pearl necklace: not so rare!

Fibromuscular dysplasia is a rare vascular disease affecting medium-caliber arteries, in particular the renal arteries and internal suprabulbar carotid arteries (can you simplify this for the general public). The prevalence of fibromuscular dysplasia in the general population is less than 0.5%, but is more frequent in expert centers that know how to evoke and diagnose it. In 5 to 10% of cases, FDM may run in families. It more specifically affects women.


Fibromuscular dysplasia is a non-atheromatous, non-inflammatory disease of medium-caliber arteries. It most often affects young subjects, with a strong female predominance. It mainly affects the renal or carotid arteries, and manifests itself as hypertension or stroke. To confirm the diagnosis, a number of imaging tests can be carried out to identify areas of narrowing or dilatation of the arteries, the most typical of which is a "string of pearls" appearance, using echo-Doppler of the renal and/or carotid arteries, angioscanner, angio-MRI or even arteriography. A therapeutic procedure is sometimes performed at the same time as the arteriogram: simple angioplasty of the artery to reopen it.

Certain signs should raise the alarm when a woman presents with arterial hypertension, especially if it is of recent onset, severe, noisy with significant headache and fatigue, and associated with a loss of potassium in the blood:
- Young age of onset < 40 years
- The onset of a cerebrovascular accident
- Occurrence of arterial dissection
- History of migraine
- Family history of arterial disease

The presence of any of these signs may suggest the onset of fibromuscular dysplasia, and the typical "string of pearls" appearance on imaging should be investigated at an expert center.



Certain factors increase the risk of developing fibromuscular dysplasia: smoking, a family history of arterial dissections or aneurysms, migraine, use of contraceptive pills containing synthetic estrogen and/or progesterone, recent pregnancy, etc.


ALERT, ANTICIPATE AND SUPPORT young women who rapidly develop hypertension, often severe and noisy! AGIR POUR LE CŒUR DES FEMMES works to inform women about certain non-atheromatous vascular diseases, such as fibrodysplastic disease. Fibrodysplastic disease is at the origin of certain types of arterial hypertension, for which it is necessary to carry out an imaging test, discuss treatment by angioplasty and, in some cases, prescribe anti-hypertensive treatment anyway, while stopping hormonal contraception with synthetic estrogens.

Key words: Fibrodysplasia - hypertension - renal artery stenosis - string of pearls - stroke - young woman - contraception with synthetic estrogen - Doppler ultrasound - angioscanner - angio-MRI - angioplasty - arteriography.

References:

1) Plouin PF, Baguet JP, Thony F, Ormezzano O, Azarine A, Silhol F, Oppenheim C, Bouhanick B, Boyer L, Persu A, Hammer F, Gosse P, Mounier-Vehier C, Le Hello C, Jeunemaitre X, Azizi M, Amar L, Chatellier G, Mousseaux E, Touzé E; ARCADIA Investigators. High Prevalence of Multiple Arterial Bed Lesions in Patients With Fibromuscular Dysplasia: The ARCADIA Registry (Assessment of Renal and Cervical Artery Dysplasia). Hypertension. 2017 Sep;70(3):652-658.
2) Silhol F, Sarlon-Bartoli G, Daniel L, Bartoli JM, Cohen S, Lepidi H, Piquet P, Bartoli MA, Vaïsse B. Intranuclear expression of progesterone receptors in smooth muscle cells of renovascular fibromuscular dysplasia: a pilot study. Ann Vasc Surg. 2015;29(4):830-5.
3) Silhol F, Radix W, Courbieres B, Cornand D, Vaïsse B, Sarlon-Bartoli G. Fibromuscular dysplasia exposes to early natural impregnation with progesterone. J Med Vasc. 2017 Dec;42(6):392-394.
4) Silhol F, Marlinge M, Guiol C, Chefrour M, Mace P, Criado C, Kipson N, Vaisse B, Vairo D, Sarlon G, Ruf J, Donnet A, Bruzzese L, Mottola G, Guieu R, Fenouillet E. Characterization of adenosine A2 receptors in peripheral blood mononuclear cells of patients with fibromuscular dysplasia. Hypertens Res. 2020 May;43(5):466-469.