Assessment of Renal and Cervical Artery DysplasIA
试验速览
- 阶段
- 不适用
- 状态
- 已完成
- 入组人数
- 499
- 试验地点
- 17
- 主要终点
- Prevalence of multisite fibromuscular dysplasia confirmed by imaging
研究概览
简要总结
ARCADIA is a national registry designed to document phenotypic and genetic traits in patients with renal and/or cervical artery fibromuscular dysplasia (FMD).
FMD is a group of arterial diseases that most commonly involve renal and carotid arteries. Patients with FMD may present with renovascular hypertension and/or with cerebrovascular symptoms. Angiographic classification includes the multifocal type and the focal type. FMD may affect one or more vascular beds and progress to more severe stenosis and to renal or cerebrovascular complications. FMD may be familial (OMIM #135580).
Our main objective is to create a FMD registry that will collect standardized information from all consenting patients diagnosed with the condition in 16 participating centers. This registry, along with a collection of leukocyte DNA, will constitute a resource for further clinical research on FMD. The first application will be the assessment of the frequency of multi-site FMD, i.e. the frequency of cervical artery FMD in patients presenting with renal artery FMD and vice-versa. The second application will be a case-control study to identify susceptibility genes for FMD.
Patients are eligible in the registry if: (a) they have renal or cervical artery FMD with either multifocal or focal lesions at CT-angiography, MR-angiography, or intra-arterial angiography; (b) they give informed consent to leukocyte DNA analysis and to the collection of bioclinical and morphologic information. Phenotypic assessment will be performed in accordance with current recommendations and best clinical practice.
Given the multicenter nature of the study and the recruitment capacity of each centre, enrollment of 500 FMD cases is expected over 5 years. This number will 1) allow an accurate estimation of the frequency of multi-site FMD: when the sample size is 500, a two-sided 95% confidence interval will extend 0.035 from the observed proportion for an expected proportion of 0.20 based on a previous report and from our unpublished data. 2) In addition to a collection of 400 renal FMD already collected at HEGP, give sufficient power for a genome-wide association study seeking for susceptibility genes
详细描述
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Background
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Definition
FMD is a group of nonatherosclerotic, noninflammatory arterial diseases that most commonly involve the renal, cervical (carotid, and vertebral) arteries. Histological classification discriminates three main subtypes, intimal, medial and perimedial, which may be associated in a single patient. 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 (unclassified FMD).
Aneurysms and dissections are considered to be complications of FMD but frequently arise in individuals with no FMD. Therefore, their presence without direct evidence of FMD does not suffice to diagnose the condition. 2. Presentations
The prevalence of symptomatic renal artery FMD is about 4/1000 and the prevalence of cervical FMD is probably half that.
研究设计
- 研究类型
- Observational
- 观察模型
- Cohort
- 时间视角
- Prospective
入排标准
- 年龄范围
- 18 Years 至 —（Adult, Older Adult）
- 性别
- All
- 接受健康志愿者
- 否
入选标准
- •Patient with renal or craniocervical fibromuscular dysplasia diagnosed during the 4 years before inclusion
- •Who understood and signed inform consent form
- •Affiliated to the French health insurance system
- •The fibromuscular dysplasia is documented by imaging (angiography, CT-angiography, MR-angiography) of less than 4 years and validated by a radiologist investigator
排除标准
- •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
结局指标
主要结局
Prevalence of multisite fibromuscular dysplasia confirmed by imaging
时间窗: Inclusion
FMD lesions discovered outside the symptomatic site
次要结局
- Clinical characteristics associated with multisite fibromuscular dysplasia(Inclusion)
- Single nucleotide polymorphisms(Inclusion)