ARCADIA (Assessment of Renal and Cervical Artery DysplasIA) is a registry designed to assess in adults with fibromuscular dysplasia (FMD) the distribution of arterial lesions and the frequency of FMD affecting at least two vascular beds (multisite FMD).Design and Method:
Patients were eligible if they had non-atherosclerotic, non-inflammatory and non-syndromic stenosing lesions affecting renal (RA) and/or cervico-encephalic (CEA) arteries and gave informed consent to undergo skull to pelvis angiographic imaging using CT-, MR-, or digital subtraction-angiography. Four vascular beds were visualized: RA, CEA, iliac and mesenteric/splenic arteries. FMD was classified as multifocal or focal, respectively, if angiography disclosed multiple or single stenoses on a given vessel segment. The diagnosis of focal and multisite FMD was confirmed centrally.Results:
486 patients (88.9% Caucasians) were included in Bordeaux, Brussels, Caen, Clermont, Grenoble, Lille, Marseille, Nancy, Paris, Saint-Etienne, Toulouse and Versailles over 3 years. RA were more frequently involved than CEA (80 vs 48% of patients, respectively). Compared with patients with focal FMD, those with multifocal FMD were older (55 ± 12 vs 38 ± 14 years, p < 0.001), less frequently smokers (18.7 vs 30.2%, p < 0.05), had more frequently bilateral RA (40.9 vs 7.5%, p < 0.0001) and CEA (39.0 vs 13.2, p = 0.0002) lesions or mesenteric/splenic lesions (18.7 vs 5.7%, p = 0.03), and more frequently exhibited multisite FMD (47.3 vs 20.8%, p = 0.0002).Conclusions:
ARCADIA is the first study in which all FMD patients had major vascular beds assessed using angiography. There was a high prevalence of bilateral and multisite lesions, particularly in patients with multifocal FMD who should be informed of the probability of having lesions on asymptomatic vascular beds. Our data suggest that FMD is a systemic rather than a local arterial disease.