Cohort Follow-up of Patients With Renal or Craniocervical Fibromuscular Dysplasia
- Conditions
- Fibromuscular Dysplasia
- Interventions
- Other: Abdominal and supra-aortic trunks vascular imagingGenetic: Blood sampling (genetic)Other: Blood sampling (biomarkers)Other: Urine sampling
- Registration Number
- NCT02961868
- Lead Sponsor
- Assistance Publique - Hôpitaux de Paris
- Brief Summary
PROFILE is a cohort study evaluating the progression of fibromuscular dysplasia lesions. This study is the prospective dimension of ARCADIA registry (ClinicalTrials.gov Identifier: NCT02884141), which aims to document phenotypic and genetic traits in patients with renal and/or cervical artery fibromuscular dysplasia.
- Detailed Description
Background
Fibromuscular dysplasia (FMD) is a group of nonatherosclerotic, noninflammatory arterial diseases that usually involve renal and carotid arteries. Patients with FMD may present with renovascular hypertension and/or with cerebrovascular symptoms. The prevalence of FMD in hypertensive patients is estimated at 4/1000. Angiographic classification includes the multifocal type, with multiple stenoses and the 'string-of-beads' appearance that is related to medial FMD, and tubular and focal types which are not clearly related to specific histological lesions. FMD may affect one or more vascular beds and progress to more severe stenosis and to renal or cerebrovascular complications. FMD appears to be familial in 10% of cases (OMIM #135580).
Renal artery FMD may progress to more severe stenosis and to renal atrophy, and/or to stenoses affecting more arteries within or outside the renal vasculature. The risk of progression as assessed from available studies was probably overestimated because documentation of progression was obtained from angiography, a procedure which is not routinely undertaken in patients with favourable clinical and biological outcomes. The disease is progressive, however, and literature stated that patients with FMD should undergo yearly duplex ultrasonography to detect progression of disease, restenosis, or loss of kidney volume.
There are very few data on prognosis of patients with symptomatic carotid or vertebral artery FMD. The risk of arterial disease progression over time is unknown. The risk of ischemic stroke ranged from 0 to about 3% per year in the few studies which assessed that issue.
Objectives
The primary objective is to estimate the incidence and risk factors for progression of FMD lesions. This will be assessed by comparison between initial and 3 years abdominal and supra-aortic trunks vascular imaging (angiography, CT-angiography or Magnetic Resonance (MR) angiography), monitoring of downstream consequences development of lesions progression and clinical events.
The secondary objectives are:
* to estimate rate of genetic polymorphism that may influence disease progression or be associated with complications
* to assess the frequency of multi-site FMD (common objective with the ARCADIA study)
* to collect standardized clinical, radiological, and biological data in patients with FMD through a national registry (common objective with the ARCADIA study)
* to organize a clinical, radiological and biological database and a biobank that will constitute a unique resource to initiate further clinical research (common objective with the ARCADIA study).
Recruitment & Eligibility
- Status
- COMPLETED
- Sex
- All
- Target Recruitment
- 340
- Patient with renal or craniocervical fibromuscular dysplasia diagnosed during the 2 years before inclusion
- The fibromuscular dysplasia is documented by imaging (angiography, CT-angiography, MR-angiography) of less than 2 years and validated by a radiologist investigator
- Patient who understood and signed inform consent form
- Affiliated to the French health insurance system
- Available for a 3 years follow-up
- Patient with renal or craniocervical atherosclerosis, or inflammatory vascular disease as dominant pathological features
- Patient with renal or craniocervical arteries dissection or aneurysm without any other evidence of fibromuscular dysplasia
- Patient under 18 or under tutorship
- Known pregnancy
Study & Design
- Study Type
- INTERVENTIONAL
- Study Design
- SINGLE_GROUP
- Arm && Interventions
Group Intervention Description Prospective cohort Blood sampling (genetic) 3 years follow-up Prospective cohort Blood sampling (biomarkers) 3 years follow-up Prospective cohort Urine sampling 3 years follow-up Prospective cohort Abdominal and supra-aortic trunks vascular imaging 3 years follow-up
- Primary Outcome Measures
Name Time Method Progression of fibromuscular dysplasia lesions confirmed by imaging 3 years
- Secondary Outcome Measures
Name Time Method Prevalence of multisite fibromuscular dysplasia confirmed by imaging Inclusion, 3 years Clinical event: renal infarction Through study completion Clinical event: revascularization procedure in a lesion site Through study completion Clinical event: ischemic stroke Through study completion Clinical event: arterial dissection in a lesion site or downstream from a lesion site Through study completion Glomerular filtration rate (GFR) Inclusion, 3 years Single nucleotide polymorphisms Inclusion Assessed by genome-wide association
Plasminogen/plasmin level Inclusion Clinical event: aneurysm rupture in a lesion site or downstream from a lesion site Through study completion Kidney height Inclusion, 3 years Matrix metalloproteinases level Inclusion
Trial Locations
- Locations (17)
CHRU de Lille hopital cardiologique
🇫🇷Lille, Hauts-de-France, France
CHRU de Lille hopital Roger-Salengro
🇫🇷Lille, Hauts-de-France, France
CHU de Caen hopital Cote de Nacre
🇫🇷Caen, Normandie, France
Cliniques universitaires Saint-Luc
🇧🇪Brussels, Brussels-Capital Region, Belgium
CHU de Nancy institut Louis-Mathieu
🇫🇷Vandeuvre-les-Nancy, Alsace-Champagne-Ardenne-Lorraine, France
CHU de Bordeaux hopital Saint-Andre
🇫🇷Bordeaux, Aquitaine-Limousin-Poitou-Charentes, France
CHU de Clermont-Ferrand hopital Gabriel-Montpied
🇫🇷Clermont-Ferrand, Auvergne-Rhone-Alpes, France
CHU de Grenoble hopital Albert-Michallon
🇫🇷La Tronche, Auvergne-Rhone-Alpes, France
Centre Hospitalier de Versailles hopital Andre Mignot
🇫🇷Le Chesnay, Ile-de-France, France
AP-HP hopital Lariboisiere
🇫🇷Paris, Ile-de-France, France
AP-HP hopital Pitie-Salpetriere
🇫🇷Paris, Ile-de-France, France
Centre hospitalier Sainte-Anne
🇫🇷Paris, Ile-de-France, France
Groupe Hospitalier Paris Saint-Joseph
🇫🇷Paris, Ile-de-France, France
AP-HP hopital Bichat-Claude-Bernard
🇫🇷Paris, Ile-de-France, France
AP-HP hopital Tenon
🇫🇷Paris, Ile-de-France, France
CHU de Toulouse hopital Rangueil
🇫🇷Toulouse, Languedoc-Roussillon-Midi-Pyrenees, France
AP-HM hopital de la Timone
🇫🇷Marseille, Provence-Alpes-Cote d'Azur, France