Fibromuscular dysplasia - results of a multicentre study in Flanders.
BACKGROUND
Fibromuscular dysplasia (FMD) is an idiopathic, non-inflammatory, non-atherosclerotic vascular disease, resulting in focal narrowing of small and medium-sized arteries. Systematic recording of clinical data in central databases as in the US and France provided new insights into FMD. The main objectives of this multicentre study were to explore the epidemiology, pattern of vascular involvement, clinical manifestations, and management of FMD patients in Flanders.


METHODS
Multicentre, retrospective registry of patients diagnosed with FMD based on medical imaging.


RESULTS
Hundred-twenty-three FMD patients (83.7 % female) were included. Mean age at FMD diagnosis was 57.3 years (SD 15.8). More than half of patients (59.5 %) were hypertensive at the time of diagnosis. Neurological complaints such as headache (26.4 %) and dizziness (23.1 %) were also frequently reported. FMD was discovered incidentally in 10 patients (8.3 %). Nearly one quarter (22.8 %) of patients experienced a cerebrovascular event. Aneurysms were found in one-fifth (20.3 %) of patients and 11.4 % had an arterial dissection. FMD affected most frequently the renal (85.3 %), carotid (74.7 %), and vertebral (39.8 %) arteries. Renovascular FMD was more prevalent in men, whereas cerebrovascular FMD was more frequent in women. Multiple affected sites were documented in 25 of 61 (41.0 %) patients, having two or more vascular beds imaged. Digital subtraction angiography was most frequently used for detecting FMD. One third (32.9 %) of patients received an interventional treatment, mainly patients with renovascular FMD (32.8 % underwent percutaneous transluminal angioplasty) and patients with an intracranial carotid aneurysm (36.4 % were treated by means of coiling).


CONCLUSIONS
Although differences existed, results of the Flemish registry were broadly in line with the US and French registries. Patient databases help to learn more about the natural history, progression, and management of FMD, based on real life clinical evidence.
1 Ghent University, Ghent, Belgium.
Marie
M
De Groote
2 Department of Nephrology and Hypertension, Universitair Ziekenhuis Brussel (VUB), Brussels, Belgium.
Patricia
P
Van der Niepen
3 Department of Neurology, Ghent University Hospital, UZ Ghent, Ghent, Belgium.
Dimitri
D
Hemelsoet
4 Centre for Medical Genetics, Ghent University Hospital, UZ Ghent, Ghent, Belgium.
Bert
B
Callewaert
5 Department of Thoracic and Vascular Surgery, Ghent University Hospital, UZ Ghent, Ghent, Belgium.
Frank
F
Vermassen
6 Department of Nephrology and Hypertension, OLV Ziekenhuis, Aalst, Belgium.
Jean-Marie
JM
Billiouw
7 Division of Nephrology and Infectious Diseases, AZ Sint-Jan, Bruges, Belgium.
An
A
De Vriese
8 Department of Nephrology, AZ Sint-Lucas, Ghent, Belgium.
Jan
J
Donck
9 Department of Cardiac and Vascular Diseases, Ghent University Hospital, UZ Ghent, Ghent, Belgium.
Tine
T
De Backer
D000328:Adult; D000368:Aged; D000783:Aneurysm / Q000000981:diagnostic imaging / Q000453:epidemiology / Q000628:therapy; D000800:Angioplasty, Balloon; D001530:Belgium / Q000453:epidemiology; D002340:Carotid Artery Diseases / Q000000981:diagnostic imaging / Q000453:epidemiology / Q000628:therapy; D002561:Cerebrovascular Disorders / Q000000981:diagnostic imaging / Q000453:epidemiology / Q000628:therapy; D003430:Cross-Sectional Studies; D004621:Embolization, Therapeutic; D005260:Female; D005352:Fibromuscular Dysplasia / Q000000981:diagnostic imaging / Q000453:epidemiology / Q000628:therapy; D006801:Humans; D033162:Incidental Findings; D008297:Male; D008875:Middle Aged; D011237:Predictive Value of Tests; D015995:Prevalence; D012042:Registries; D012189:Retrospective Studies; D017678:Sex Distribution; D016896:Treatment Outcome
Fibromuscular dysplasia; cerebrovascular; medical imaging; registries; renovascular