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Systemic sclerosis (SSc) is a rare form of connective tissue disease characterized by vascular involvement and the intensity of fibrosis. The lack of available treatment is largely due to the very fragmented understanding of the pathophysiology of SSc. However, one of the keys to conducting quality research on this disease remains the development of well-documented patient cohorts with reliable biological samples. The main objective of this cohort is to study the natural progression of SSc in a cohort of patients followed over 5 years.
Systemic sclerosis (SSc) is a rare form of connective tissue disease characterized by vascular involvement and the intensity of fibrosis. Its prevalence and incidence are difficult to assess, however, in France, a population survey conducted in Seine-St-Denis calculated a prevalence of 161 cases per million inhabitants.
The pathophysiology of SSc, the exact etiology of which remains unknown, involves an interaction between genetic and environmental factors. Its evolution can impact the aesthetic, functional and even vital prognosis of the affected patient.Within the analysis of SSc pathophysiology, a " very early systemic sclerosis " form of disease has been defined according to the presence of Raynaud's phenomenon and auto-antibodies in blood sample (ACAN positivity (≥1/160) with anti-Scl70, anti-centromere or anti-ARNPolIII specificity).
At present, no treatment to control this disease is available. The lack of available treatment is largely due to the very fragmented understanding of the pathophysiology of SSc. However, one of the keys to research remains the development of well-documented patient cohorts with quality biological samples. The investigators had the opportunity to start a major work on this plan with the VISS study (Vasculopathy and Inflammation in Systemic Scleroderma study) in 2012 as part of a project promoted by the University Hospital of Bordeaux (NCT02562079). This project has paved the way for many local, national and international collaborations. It has made it possible to structure and federate various partners of the Bordeaux University Hospital around translational research on SSc.
The investigators wish to continue our research and collaborations by further strengthening our expertise in the collection of rare and valuable biological samples for this disease.
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| Label | Type | Description | Intervention Names |
|---|---|---|---|
| subjects SSc diagnosed | Experimental | Patient with systemic scleroderma according to the American College of Rheumatology (ACR) / EULAR 2013 criteria |
|
| Name | Type | Description | Arm Group Labels | Other Names |
|---|---|---|---|---|
| Blood samples | Biological | 62 ml whole blood for Peripheral blood mononuclear cell (PBMC) and monocytes isolation |
|
| Measure | Description | Time Frame |
|---|---|---|
| Change of the main clinical characteristics of scleroderma patients | Worsening of the SSc according to the onset of a renal crisis (according to arterial hypertension > 150/85 mm Hg ), a pulmonary arterial hypertension (identified with a right heart catheterization), or an interstitial lung disease (identified with a chest CT-scan). | At baseline (Day 0) and 60 months after baseline |
| Measure | Description | Time Frame |
|---|---|---|
| Proportion of pulmonary arterial hypertension diagnosis in SSc patients | At baseline (Day 0) and 60 months after baseline | |
| Proportion of interstitial lung disease diagnosis in SSc patients | At baseline (Day 0) and 60 months after baseline |
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Inclusion Criteria:
Exclusion Criteria:
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| Name | Role | Phone | Extension | |
|---|---|---|---|---|
| Marie-Elise TRUCHETET, MD, PhD | Contact | 05.56.79.55.56 | +33 | marie-elise.truchetet@chu-bordeaux.fr |
| Thomas BARNETCHE, PhD | Contact | 05.57.82.04.93 | +33 | thomas.barnetche@chu-bordeaux.fr |
| Name | Affiliation | Role |
|---|---|---|
| Marie-Elise TRUCHETET, MD, PhD | University Hospital, Bordeaux | Principal Investigator |
| Facility | Status | City | State | ZIP | Country | Contacts |
|---|---|---|---|---|---|---|
| CHU de Bordeaux - service de rhumatologie | Recruiting | Bordeaux | France |
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| ID | Term |
|---|---|
| D045743 | Scleroderma, Diffuse |
| D012595 | Scleroderma, Systemic |
| ID | Term |
|---|---|
| D003240 | Connective Tissue Diseases |
| D017437 | Skin and Connective Tissue Diseases |
| D012871 | Skin Diseases |
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| ID | Term |
|---|---|
| D001800 | Blood Specimen Collection |
| D001706 | Biopsy |
| ID | Term |
|---|---|
| D013048 | Specimen Handling |
| D019411 | Clinical Laboratory Techniques |
| D019937 | Diagnostic Techniques and Procedures |
| D003933 | Diagnosis |
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| Biopsy | Other | Skin biopsies only for volunteers among patients |
|
| Bronchoalveolar samples | Other | 50 ml of bronchoalveolar samples if pulmonary flare requires this type of exploration only for volunteers among patients |
|
| Biopsy | Other | Digestives biopsies if requires this type of exploration in the standard of care only for volunteers among patients |
|
| Proportion of renal crisis diagnosis in SSc patients | At baseline (Day 0) and 60 months after baseline |
| Mean of Rodnan score for the evaluation of disease activity for SSc patients, with higher values mean higher disease activity. | (Min value: 0 - Max value: 51) | At baseline (Day 0) and 60 months after baseline |
| Mean of Diffusing capacity (DLCO) for the evaluation of disease activity for SSc patients | At baseline (Day 0) and 60 months after baseline |
| Mean of Forced vital capacity (FVC) for the evaluation of disease activity for SSc patients | At baseline (Day 0) and 60 months after baseline |
| Proportion of therapeutic strategies set up for SSc patients | At baseline (Day 0) and 60 months after baseline |
| D011677 | Punctures |
| D013514 | Surgical Procedures, Operative |
| D008919 | Investigative Techniques |
| D003581 | Cytodiagnosis |
| D003584 | Cytological Techniques |
| D003949 | Diagnostic Techniques, Surgical |