Cohort of Patients With Systemic Sclerosis and Associated Biological Collection Within the Framework of the RESO Reference Centre for Rare Systemic Autoimmune Diseases
试验速览
- 阶段
- 不适用
- 状态
- 招募中
- 发起方
- 入组人数
- 500
- 试验地点
- 1
- 主要终点
- Change of the main clinical characteristics of scleroderma patients
研究概览
简要总结
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.
详细描述
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.
研究设计
- 研究类型
- Interventional
- 分配方式
- Na
- 干预模型
- Single Group
- 主要目的
- Other
- 盲法
- None
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 over 18 years old
- •Patient with systemic scleroderma according to the ACR/EULAR 2013 criteria, or with a " very early systemic sclerosis " defined by 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).
- •Person affiliated or benefiting from a social security scheme.
- •Free, informed and written consent signed by the participant and the investigator (no later than the day of inclusion and prior to any review required by the research)
排除标准
- •Pregnant or breastfeeding woman
- •Patient under guardianship, curatorship or any other legal protection regime
研究组 & 干预措施
subjects SSc diagnosed
Patient with systemic scleroderma according to the American College of Rheumatology (ACR) / EULAR 2013 criteria
干预措施: Blood samples (Biological)
subjects SSc diagnosed
Patient with systemic scleroderma according to the American College of Rheumatology (ACR) / EULAR 2013 criteria
干预措施: Biopsy (Other)
subjects SSc diagnosed
Patient with systemic scleroderma according to the American College of Rheumatology (ACR) / EULAR 2013 criteria
干预措施: Bronchoalveolar samples (Other)
结局指标
主要结局
Change of the main clinical characteristics of scleroderma patients
时间窗: At baseline (Day 0) and 60 months after baseline
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).
次要结局
- Proportion of pulmonary arterial hypertension 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.(At baseline (Day 0) and 60 months after baseline)
- Proportion of renal crisis diagnosis in 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)
- Proportion of interstitial lung disease diagnosis in SSc patients(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)