Department of Cardiology, Sud-Île-de-France Hospital Group, Melun, France
Received Date: 02/03/2025; Published Date: 07/04/2025
*Corresponding author: Soumare Ahamadou, Cardiology Department, Sud-Île-de-France Hospital Group, Melun, France
Fibromuscular dysplasia (FMD) is an idiopathic systemic arteriopathy that is non-atherosclerotic and non-inflammatory, affecting small and medium-sized arteries, most frequently the renal and carotid arteries, but it can involve all arteries. It is a rare disease that primarily affects women and can be indicative of secondary hypertension due to renovascular stenosis, ischemic stroke, or arterial dissection when it affects the carotid and vertebral arteries. We report here a case of fibromuscular dysplasia of the renal arteries in a 66-year-old woman.
Keywords: Fibromuscular dysplasia; Hypertension; Renal artery; Carotid artery
Fibromuscular dysplasia is a rare disease first described by Leadbetter and Burkland in 1938. It more frequently affects young or middle-aged women with a prevalence of about 4% in the general population. All arteries can be affected, primarily the renal and carotid arteries, which can be responsible for renovascular hypertension or ischemic stroke. The presence of multifocal stenoses and dilation with a "string of beads" appearance allows for a characteristic diagnosis in the majority of cases.
This is a 66-year-old female patient with no significant medical history, hospitalized in the Cardiology Department of the Sud-Île-de-France Hospital Group in Melun for recently discovered hypertension associated with headaches and dizziness.
Clinical examination revealed systolic-diastolic hypertension at 210/120 mmHg without anisotension. There was left ventricular hypertrophy on transthoracic echocardiography. Laboratory tests were unremarkable.
As part of the etiological assessment, a CT angiography of the renal arteries showed caliber irregularity in both renal arteries compatible with signs of angiodysplasia.
Given this suggestive context of fibromuscular dysplasia, further explorations were completed with a CT angiography of the supra-aortic trunks, which revealed dysplastic-appearing internal carotids; CT angiography of the aorta and lower limbs and a coronary CT scan returned without significant anomalies.
The diagnosis of fibromuscular dysplasia with bilateral carotid involvement and renal arteries possibly responsible for severe hypertension was retained, and the patient was transferred to the Hypertension Center at Georges Pompidou Hospital in Paris.
Fibromuscular dysplasia is a rare disease. The prevalence of renal FMD is 0.4%, while the prevalence of cranio-cervical FMD is much lower, at 0.1%. A series of autopsies from the Mayo Clinic reported a prevalence of 1.1% for renal FMD, while the prevalence among kidney donors who underwent angiography ranged from 2% to 6.6%. Among older hypertensive patients who underwent renal angiography, a prevalence of 5.8% was noted in the Cardiovascular Outcomes in Renal Atherosclerotic
Lesions (CORAL) trial, and in 2020, Rana estimated the prevalence of FMD at 12 per 100,000 people in the American population. However, the prevalence appears to be higher but underdiagnosed.
The diagnosis of FMD is suggested in cases of severe hypertension in women and relies on imaging (CT angiography as the first-line, Angio ARM) or angiography. The presence of at least one focal or multifocal arterial lesion is required to make the diagnosis. However, FMD can also be associated with non-specific lesions, such as aneurysms, dissections, and arterial tortuosity. Renal, cerebrovascular, and cervical involvements are the most common.
Therapeutic management has not been the subject of randomized studies due to the rarity of the disease. There are no comparative studies between medical treatment, interventional (percutaneous revascularization), and surgery. However, it is recommended to discuss therapeutic strategies on a case-by-case basis in multidisciplinary consultation meetings (MCM) involving specialists in hypertension, nephrologists, cardiologists, and interventionalists, as was the case for our patient after discussion with the hypertensologists at the Hypertension Center of Georges Pompidou Hospital in Paris where she was transferred.
Figure 1: Angio scanner Artère rénale.
Figure 2: Angio scanner Carotides droite et gauche.
Fibromuscular dysplasia is a rare and underdiagnosed condition. It primarily affects the renal and carotid arteries. The diagnosis should be considered in young and middle-aged hypertensive women. Arterial imaging via CT angiography remains the gold standard for confirming the diagnosis. In the absence of clear recommendations, therapeutic management—whether medical, interventional, or surgical—should be handled on a case-by-case basis with expert centers.