[PP.09.05] the Polish Registry for Fibromuscular Dysplasia (Arcadia-Pol Study) – Distribution of Vascular Bed Involvement and Complications in Patients With Fibromuscular Dysplasia

Fibromuscular dysplasia: its various phenotypes in everyday practice in 2021

“…The typical clinical presentation of renal FMD is a young to middle-aged, Caucasian woman with arterial hypertension [9][10][11][12]15]. While the prevalence of FMD in the general population is unknown, studies from living kidney donor candidates indicate a prevalence of renal FMD of about 3.3% [24].…”

“…Severe forms have been reported, eventually resulting in hemicolectomy, multi-organ failure and eventually death. Acute ischemia and/or infarction is usually due to arterial dissection or embolism from an aneurysm causing acute abdominal pain and potentially shock [3, 12,13]. Patients with VA-FMD are more likely to have aneurysms and dissections compared to patients with FMD without visceral involvement [76].…”

“…Aneurysms, dissections, and arterial tortuosity are also frequent manifestations of the disease, but these lesions can only be attributed to FMD in the presence of a typical string-ofbeads or focal stenosis, as these lesions have been described in other vascular diseases as well [3,[6][7][8]. Renal and cerebrovascular arteries are most frequently involved, but all other mediumsized arteries may be affected [3,[9][10][11][12][13][14]. Involvement of >1 vascular bed, i.e.…”

“…Involvement of >1 vascular bed, i.e. multivessel FMD, is seen in a substantial proportion of patients, ranging from 30% to 57% [3,[9][10][11][12][13]15].…”

“…Recent prospective registries and ongoing research have substantially improved our knowledge and understanding of the disease's clinical manifestations, epidemiology, natural history and genetics [9][10][11][12]19]. FMD is no longer considered a rare cause of renovascular hypertension in young women, but rather appears as a systemic arterial disease, which may occur in both sexes at any age and with a wide range of manifestations.…”