Ignite Creation Date:
2024-05-06 @ 3:56 PM
Last Modification Date:
2024-10-26 @ 1:59 PM
Study NCT ID:
NCT04804683
Status:
RECRUITING
Last Update Posted:
2022-05-05
First Post:
2021-03-15
Brief Title:
EuropeanInternational FMD Registry and Initiative
Sponsor:
Cliniques universitaires Saint-Luc- Université Catholique de Louvain
Organization:
Cliniques universitaires Saint-Luc- Université Catholique de Louvain
Study Overview
Official Title:
The EuropeanInternational FMD Registry and Initiative FEIRI a Prospective Study
Status:
RECRUITING
Status Verified Date:
2022-05
Last Known Status:
None
Delayed Posting:
No
If Stopped, Why?:
Not Stopped
Has Expanded Access:
False
If Expanded Access, NCT#:
N/A
Has Expanded Access, NCT# Status:
N/A
Acronym:
FEIRI
Brief Summary:
The main objectives of FEIRI are
i To describe the demographic and arterial characteristics of FMD and related diseases at a global scale and according to countries andor ethnic origin
ii To evaluate the incidence and predictors of novel FMD lesions and complications
iii To explore the commonalities and differences between FMD SCAD and so-called atypical FMD patients with multiple dissections andor aneurysms without string-of-beads focal stenosis or evidence of inherited arteriopathy
iv To contribute to the unravelling of genetic proteomic and molecular mechanisms underlying FMD and related diseases
Participation to the FEIRI study implies
i Collection of demographic and standard-of-care clinical data both retrospectively from the diagnosis of FMD to signature of the informed consent and prospectively on the occasion of standard-of-care follow-up
ii Optional participation to a biobank implying collection of blood urine and in rare cases of intervention tissue samples for genomic and proteomic analysis and identification of diagnostic and prognostic biomarkers of FMD
Participants will be enrolled in centres from over 20 countries in Europe and beyond
i To describe the demographic and arterial characteristics of FMD and related diseases at a global scale and according to countries andor ethnic origin
ii To evaluate the incidence and predictors of novel FMD lesions and complications
iii To explore the commonalities and differences between FMD SCAD and so-called atypical FMD patients with multiple dissections andor aneurysms without string-of-beads focal stenosis or evidence of inherited arteriopathy
iv To contribute to the unravelling of genetic proteomic and molecular mechanisms underlying FMD and related diseases
Participation to the FEIRI study implies
i Collection of demographic and standard-of-care clinical data both retrospectively from the diagnosis of FMD to signature of the informed consent and prospectively on the occasion of standard-of-care follow-up
ii Optional participation to a biobank implying collection of blood urine and in rare cases of intervention tissue samples for genomic and proteomic analysis and identification of diagnostic and prognostic biomarkers of FMD
Participants will be enrolled in centres from over 20 countries in Europe and beyond
Detailed Description:
Fibromuscular dysplasia FMD is an idiopathic segmental nonatherosclerotic and noninflammatory disease of the musculature of arterial walls leading to stenosis of small- and medium-sized arteries FMD can be classified in multifocal FMD characterized by alternation of stenosis and dilations string-of-beads and focal FMD corresponding to a solitary narrowing While for long FMD was considered as a rare cause of renovascular disease mostly affecting young women during the last decades joint international efforts have led to a thorough reappraisal of the disease In summary i FMD is no more considered as a truly rare disease ii FMD may be also diagnosed in men 10-20 and at all ages iii beyond arterial stenosis FMD is often associated with dissections aneurysms and arterial tortuosity iv FMD is not restricted to renal arteries and often affects two or more arterial beds iv multifocal FMD lesions are frequent in patients with Spontaneous Coronary Artery Dissection SCAD
Despite traditional views on the role of female hormones mechanical factors and smoking the pathophysiology of FMD remains largely unknown In the last decade research has been focused on genetic dissection of the disease and identification of biomarkers Recent advances include i identification of an association between FMD and an intronic variant of the Phosphatase and actin regulator 1 PHACTR1 gene ii identification of rare mutations in several genes such as Prostaglandin I2 Receptor PTGIR and Collagen type V alpha 1 chain COL5A1 genes iii documentation of mild Connective-Tissue Disease-like features and increased levels of Transforming Growth Factor-beta in patients with FMD iv identification of a tentative proteo-genomic signature of the disease All elements are thus in place to further dissect the pathophysiology of FMD and related diseases refine clinical characterization identify predictors of complications and improve screening management and follow-up
Unravelling the clinical characteristics genetic and molecular basis of FMD nevertheless requires large numbers of well characterized patients In order to address these challenges and generate new evidence pertaining to FMD and associated diseases we aimed to create an overarching resource and study named the EuropeanInternational FMD Registry and Initiative acronym FEIRI
The objectives of FEIRI are
To describe the demographic and arterial characteristics of FMD and related diseases at a global scale and according to countries andor ethnic origin
To identify environmental hormonal factors and exposures associated with the onset and progression of FMD
To evaluate the incidence and predictors of novel FMD lesions and complications
To provide evidence-based algorithms for the diagnosis and optimal management and follow-up of patients with FMD
To explore the commonalities and differences between FMD SCAD and so-called atypical FMD patients with multiple dissections andor aneurysms without string-of-beads focal stenosis or evidence of inherited arteriopathy
To contribute to the unravelling of genetic proteomic and molecular mechanisms underlying FMD and related diseases
Participation to the FEIRI study implies
Collection of demographic and standard-of-care clinical data both retrospectively from the diagnosis of FMD to signature of the informed consent and prospectively on the occasion of standard-of-care follow-up
Optional participation both for centres and patients to a biobank implying collection of blood urine and in rare cases of intervention tissue samples for genomic and proteomic analysis and identification of diagnostic and prognostic biomarkers of FMD
Participants will be enrolled in centres from over 20 countries in Europe and beyond
Despite traditional views on the role of female hormones mechanical factors and smoking the pathophysiology of FMD remains largely unknown In the last decade research has been focused on genetic dissection of the disease and identification of biomarkers Recent advances include i identification of an association between FMD and an intronic variant of the Phosphatase and actin regulator 1 PHACTR1 gene ii identification of rare mutations in several genes such as Prostaglandin I2 Receptor PTGIR and Collagen type V alpha 1 chain COL5A1 genes iii documentation of mild Connective-Tissue Disease-like features and increased levels of Transforming Growth Factor-beta in patients with FMD iv identification of a tentative proteo-genomic signature of the disease All elements are thus in place to further dissect the pathophysiology of FMD and related diseases refine clinical characterization identify predictors of complications and improve screening management and follow-up
Unravelling the clinical characteristics genetic and molecular basis of FMD nevertheless requires large numbers of well characterized patients In order to address these challenges and generate new evidence pertaining to FMD and associated diseases we aimed to create an overarching resource and study named the EuropeanInternational FMD Registry and Initiative acronym FEIRI
The objectives of FEIRI are
To describe the demographic and arterial characteristics of FMD and related diseases at a global scale and according to countries andor ethnic origin
To identify environmental hormonal factors and exposures associated with the onset and progression of FMD
To evaluate the incidence and predictors of novel FMD lesions and complications
To provide evidence-based algorithms for the diagnosis and optimal management and follow-up of patients with FMD
To explore the commonalities and differences between FMD SCAD and so-called atypical FMD patients with multiple dissections andor aneurysms without string-of-beads focal stenosis or evidence of inherited arteriopathy
To contribute to the unravelling of genetic proteomic and molecular mechanisms underlying FMD and related diseases
Participation to the FEIRI study implies
Collection of demographic and standard-of-care clinical data both retrospectively from the diagnosis of FMD to signature of the informed consent and prospectively on the occasion of standard-of-care follow-up
Optional participation both for centres and patients to a biobank implying collection of blood urine and in rare cases of intervention tissue samples for genomic and proteomic analysis and identification of diagnostic and prognostic biomarkers of FMD
Participants will be enrolled in centres from over 20 countries in Europe and beyond
Study Oversight
Has Oversight DMC:
None
Is a FDA Regulated Drug?:
False
Is a FDA Regulated Device?:
False
Is an Unapproved Device?:
None
Is a PPSD?:
None
Is a US Export?:
None
Is an FDA AA801 Violation?:
None