Fibromuscular dysplasia (FMD) is an idiopathic, segmental, noninflammatory, nonatherosclerotic vascular disease, which results in arterial narrowing of small and medium-sized vessels. The last years new perspectives of the disease have opened up thanks to the systematically recording of FMD patient's data in central databases in the US and France. The main objectives of this multicentre study were to discover the epidemiology, vascular involvement, clinical manifestations and management of FMD patients in Flanders. Later on, these results will be compared to those of the French & US Registry to discover new insights about the disease.Design and method:
Multicentre study at 5 different hospitals in Flanders. 123 FMD patients were included in the database. Patients were eligible for enrolment if they were adults (18+ years old), diagnosis of FMD was confirmed using medical imaging and permission was given by written informed consent.Results:
Patients were on average 57.3 years (SD 15.8) when FMD was first diagnosed. 83.7% were female. Arterial hypertension (38.5%) was the most frequent symptom leading to diagnosis of FMD. Neurological complaints such as headache (26.4%), vertigo and/or balancing problems (23.1%) and a cervical bruit 17.5%) were also common. Ten patients (8.3%) had no symptoms at the time of diagnosis. Vascular events and complications as a result of FMD occurred in a minority of patients (21.1%). Cerebrovascular involvement (81.8%) was nearly as frequent as renovascular FMD (85.3%). In 25 of 60 (41.7%) patient having two or more vascular beds imaged, co-existent FMD was found in at least two vascular beds. Digital subtraction angiography was the preferred technique for detecting FMD related lesions. The majority of the patients was pharmacologically treated (86.3%), 25.9% of them underwent an endovascular treatment.Conclusions:
This study confirmed the finding that FMD is a disorder of mostly middle-aged people, but can be found in any age group. The carotid/vertebral arteries were nearly as frequently involved as the renovascular bed. Symptoms as a result of FMD were highly variable, with FMD incidentally discovered in a minority of all patients.