NCT04265144

Cohort of Patients With Systemic Sclerosis Within the Framework of the RESO Reference Centre

Cohort of Patients With Systemic Sclerosis and Associated Biological Collection Within the Framework of the RESO Reference Centre for Rare Systemic Autoimmune Diseases

• Status: Recruiting
• Phase: N/A
• Study type: Interventional
• Enrollment: 500 (estimated)
• Sponsor: University Hospital, Bordeaux · Academic / Other
• Sex: All
• Age: 18 Years
• Healthy volunteers: Not accepted

Summary

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.

Detailed description

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.

Conditions

• Scleroderma
• Systemic Sclerosis

Interventions

Type	Name	Description
BIOLOGICAL	Blood samples	62 ml whole blood for Peripheral blood mononuclear cell (PBMC) and monocytes isolation
OTHER	Biopsy	Skin biopsies only for volunteers among patients
OTHER	Bronchoalveolar samples	50 ml of bronchoalveolar samples if pulmonary flare requires this type of exploration only for volunteers among patients
OTHER	Biopsy	Digestives biopsies if requires this type of exploration in the standard of care only for volunteers among patients

Timeline

• Start date: 2020-06-08
• Primary completion: 2034-06-01
• Completion: 2034-06-01
• First posted: 2020-02-11
• Last updated: 2025-09-04

Locations

1 site across 1 country: France