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

University Hospital of Bordeaux

Status: Enrolling

Conditions: Scleroderma; Systemic Sclerosis

Treatments: Other: Biopsy; Biological: Blood samples; Other: Bronchoalveolar samples

Study type: Interventional

Funder types: Other

Identifiers

NCT04265144

CHUBX 2019/42

Details and patient eligibility

About

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.

Full 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.

Enrollment: 500 estimated patients

Sex: All

Ages: 18+ years old

Volunteers: No Healthy Volunteers

Inclusion criteria

• 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)

Exclusion criteria

• Pregnant or breastfeeding woman
• Patient under guardianship, curatorship or any other legal protection regime

Trial design

Primary purpose: Other

Allocation: N/A

Interventional model: Single Group Assignment

Masking: None (Open label)

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500 participants in 1 patient group

subjects SSc diagnosed

Experimental group

Description:

Patient with systemic scleroderma according to the American College of Rheumatology (ACR) / EULAR 2013 criteria

Treatment:

Other: Biopsy

Other: Bronchoalveolar samples

Biological: Blood samples

Other: Biopsy