Systematic and Multidisciplinary Evaluation of Fibromuscular Dysplasia Patients Reveals High Prevalence of Previously Undetected Fibromuscular Dysplasia Lesions and Affects Clinical Decisions

Abstract

Fibromuscular dysplasia (FMD), regarded as a generalized vascular disease, may affect all vascular beds and may result in arterial stenosis, occlusion, aneurysm, or dissection. It has been proposed to systematically evaluate all vascular beds in patients with FMD, regardless of initial FMD involvement. However, the impact of this approach on clinical decisions and on management is unknown. Within the prospective ARCADIA-POL study (Assessment of Renal and Cervical Artery Dysplasia–Poland), we evaluated 232 patients with FMD lesions confirmed in at least one vascular bed, out of 343 patients included in the registry. All patients underwent a detailed clinical evaluation including computed tomography angiography of intracranial and cervical arteries, as well as computed tomography angiography of the abdominal aorta, its branches, and upper and lower extremity arteries. In the study group, FMD lesions were most frequently found in renal arteries (87.5%). FMD was also found in cerebrovascular (24.6%), mesenteric (13.8%), and upper (3.0%) and lower extremity (9.9 %) arteries. Newly diagnosed FMD lesions were found in 34.1% of the patients, and previously undetected vascular complications were found in 25% of the patients. Among all FMD patients included in the study, one out of every 4 evaluated patients qualified for interventional treatment due to newly diagnosed FMD lesions or vascular complications. The ARCADIA-POL study shows for the first time that the systematic and multidisciplinary evaluation of patients with FMD based on a whole-body computed tomography angiography scan has an impact on their clinical management. This proved the necessity of the systematic evaluation of all vascular beds in patients with FMD, regardless of initial FMD involvement.

Fibromuscular Dysplasia/Carotid Web in Angio-CT Imaging: A Rare Cause of Ischemic Stroke

“…Haussen et al reported the recurrence rate of 29% in patients treated with pharmacotherapy, whereas no recurrent stroke/TIA was observed in stented patients [ 22 ]. If CaW is found, looking for FMD in other locations should be considered [ 26 ].…”

The diagnosis and management of spontaneous coronary artery dissection — expert opinion of the Association of Cardiovascular Interventions (ACVI) of Polish Cardiac Society

“…FMD may also be more frequent in SCAD patients with a higher coronary tortuosity score [65]. In the ARCADIA-POL study, including 232 patients with FMD, systematic evaluation including "head-to-pelvis" computed tomography angiography resulted in detection of new FMD lesions in 34.1% of patients and previously undiagnosed vascular complications in 25% of subjects [73]. These findings require confirmation in SCAD-cohort observational studies.…”

Fibromuscular dysplasia: its various phenotypes in everyday practice in 2021

“…Cervical (carotid or vertebral) artery dissection is a frequent event of FMD (Figure 2D). The prevalence varies between 9% and 39.5%, and is highest among patients with a neurological presentation leading to diagnosis of FMD [6,10,11,16,53,59,60]. In the US FMD registry, the prevalence of carotid artery dissection is 3 to 4 times higher than vertebral artery dissection [6,10].…”

“…The prevalence of FMD in the general population is unknown because this disease is often clinically silent. FMD may be diagnosed during work-up of clinical symptoms and/or signs, but can also be discovered incidentally during imaging tests performed for other reasons [3,16].…”