Clinical Trial: Cohort Follow-up of Patients With Renal or Craniocervical Fibromuscular Dysplasia

Study Status: Active, not recruiting
Recruit Status: Active, not recruiting
Study Type: Interventional

Official Title: PROgression of FIbromuscular LEsions

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 Summary:

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 inciden
Sponsor: Assistance Publique - Hôpitaux de Paris

Current Primary Outcome: Progression of fibromuscular dysplasia lesions confirmed by imaging [ Time Frame: 3 years ]

Original Primary Outcome: Same as current

Current Secondary Outcome:

  • Glomerular filtration rate (GFR) [ Time Frame: Inclusion, 3 years ]
  • Kidney height [ Time Frame: Inclusion, 3 years ]
  • Clinical event: revascularization procedure in a lesion site [ Time Frame: Through study completion ]
  • Clinical event: renal infarction [ Time Frame: Through study completion ]
  • Clinical event: ischemic stroke [ Time Frame: Through study completion ]
  • Clinical event: arterial dissection in a lesion site or downstream from a lesion site [ Time Frame: Through study completion ]
  • Clinical event: aneurysm rupture in a lesion site or downstream from a lesion site [ Time Frame: Through study completion ]
  • Prevalence of multisite fibromuscular dysplasia confirmed by imaging [ Time Frame: Inclusion, 3 years ]
  • Single nucleotide polymorphisms [ Time Frame: Inclusion ]
    Assessed by genome-wide association
  • Plasminogen/plasmin level [ Time Frame: Inclusion ]
  • Matrix metalloproteinases level [ Time Frame: Inclusion ]


Original Secondary Outcome: Same as current

Information By: Assistance Publique - Hôpitaux de Paris

Dates:
Date Received: November 7, 2016
Date Started: November 2009
Date Completion: December 2017
Last Updated: November 16, 2016
Last Verified: November 2016