Clinical trial NCT02961868: Cohort Follow-up of Patients With Renal or Craniocervical Fibromuscular Dysplasia
Official title: PROgression of FIbromuscular LEsions
Study type: INTERVENTIONAL
Overall status: COMPLETED
Phase: NA
Conditions: Fibromuscular Dysplasia
Keywords: Renal Artery Obstruction
Carotid Artery Diseases
Genetic Association Studies
Lead sponsor: Assistance Publique - Hôpitaux de Paris
Sponsor class: OTHER
Collaborators: name: Fondation de Recherche sur l'Hypertension Artérielle
First posted: 2016-11-11
Enrollment: 340
Sex: ALL
Minimum age (years): 18
Standard ages: ADULT
OLDER_ADULT
Accepts healthy volunteers: no
Allocation: NA
Intervention model: SINGLE_GROUP
Primary purpose: DIAGNOSTIC

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).

Eligibility criteria: Inclusion Criteria:

* 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

Exclusion Criteria:

* 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

Primary outcomes:
measure: Progression of fibromuscular dysplasia lesions confirmed by imaging
timeFrame: 3 years
Secondary outcomes:
measure: Glomerular filtration rate (GFR)
timeFrame: Inclusion, 3 years
measure: Kidney height
timeFrame: Inclusion, 3 years
measure: Clinical event: revascularization procedure in a lesion site
timeFrame: Through study completion
measure: Clinical event: renal infarction
timeFrame: Through study completion
measure: Clinical event: ischemic stroke
timeFrame: Through study completion
measure: Clinical event: arterial dissection in a lesion site or downstream from a lesion site
timeFrame: Through study completion
measure: Clinical event: aneurysm rupture in a lesion site or downstream from a lesion site
timeFrame: Through study completion
measure: Prevalence of multisite fibromuscular dysplasia confirmed by imaging
timeFrame: Inclusion, 3 years
measure: Single nucleotide polymorphisms
timeFrame: Inclusion
measure: Plasminogen/plasmin level
timeFrame: Inclusion
measure: Matrix metalloproteinases level
timeFrame: Inclusion
Other outcomes:
Locations:
city: Brussels
country: Belgium
facility: Cliniques universitaires Saint-Luc
state: Brussels-Capital Region
city: Vandeuvre-les-Nancy
country: France
facility: CHU de Nancy institut Louis-Mathieu
state: Alsace-Champagne-Ardenne-Lorraine
city: Bordeaux
country: France
facility: CHU de Bordeaux hopital Saint-Andre
state: Aquitaine-Limousin-Poitou-Charentes
city: Clermont-Ferrand
country: France
facility: CHU de Clermont-Ferrand hopital Gabriel-Montpied
state: Auvergne-Rhone-Alpes
city: La Tronche
country: France
facility: CHU de Grenoble hopital Albert-Michallon
state: Auvergne-Rhone-Alpes
city: Lille
country: France
facility: CHRU de Lille hopital cardiologique
state: Hauts-de-France
city: Lille
country: France
facility: CHRU de Lille hopital Roger-Salengro
state: Hauts-de-France
city: Le Chesnay
country: France
facility: Centre Hospitalier de Versailles hopital Andre Mignot
state: Ile-de-France
city: Paris
country: France
facility: AP-HP hopital Lariboisiere
state: Ile-de-France
city: Paris
country: France
facility: AP-HP hopital Pitie-Salpetriere
state: Ile-de-France
city: Paris
country: France
facility: Centre hospitalier Sainte-Anne
state: Ile-de-France
city: Paris
country: France
facility: Groupe Hospitalier Paris Saint-Joseph
state: Ile-de-France
city: Paris
country: France
facility: AP-HP hopital Bichat-Claude-Bernard
state: Ile-de-France
city: Paris
country: France
facility: AP-HP hopital Tenon
state: Ile-de-France
city: Toulouse
country: France
facility: CHU de Toulouse hopital Rangueil
state: Languedoc-Roussillon-Midi-Pyrenees
city: Caen
country: France
facility: CHU de Caen hopital Cote de Nacre
state: Normandie
city: Marseille
country: France
facility: AP-HM hopital de la Timone
state: Provence-Alpes-Cote d'Azur
Study officials:
affiliation: Assistance Publique - Hôpitaux de Paris
name: Pierre-François Plouin, MD
role: PRINCIPAL_INVESTIGATOR
References: