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

Systemic Sclerosis Clinical Trial

— SCLERESO  

Official title:

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

Clinical Trial Details — Status: Recruiting

Administrative data

• NCT number: NCT04265144
• Other study ID #: CHUBX 2019/42
• Status: Recruiting
• Phase: N/A
• Start date: June 8, 2020
• Est. completion date: June 2030

Study information

• Verified date: January 2024
• Source: University Hospital, Bordeaux
• Contact: Marie-Elise TRUCHETET, MD, PhD
• Phone: 05.56.79.55.56
• Email: marie-elise.truchetet[@]chu-bordeaux.fr
• Is FDA regulated: No
• Study type: Interventional

Clinical Trial 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.

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.

Recruitment information / eligibility

• Status: Recruiting
• Enrollment: 500
• Est. completion date: June 2030
• Est. primary completion date: June 2030
• Accepts healthy volunteers: No
• Gender: All
• Age group: 18 Years and older

Eligibility

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

Related Conditions & MeSH terms

• Scleroderma
• Scleroderma, Diffuse
• Scleroderma, Systemic
• Sclerosis
• Systemic Sclerosis

Intervention

Biological:
• Blood samples
62 ml whole blood for Peripheral blood mononuclear cell (PBMC) and monocytes isolation

Other:
• Biopsy
Skin biopsies
• Bronchoalveolar samples
50 ml of bronchoalveolar samples if pulmonary flare requires this type of exploration

Locations

Country	Name	City	State
France	CHU de Bordeaux - service de rhumatologie	Bordeaux

Sponsors (1)

Lead Sponsor	Collaborator
University Hospital, Bordeaux

Country where clinical trial is conducted

France, 

Outcome

Type	Measure	Description	Time frame	Safety issue
Primary	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
Secondary	Proportion of pulmonary arterial hypertension diagnosis in SSc patients		At baseline (Day 0) and 60 months after baseline
Secondary	Proportion of interstitial lung disease diagnosis in SSc patients		At baseline (Day 0) and 60 months after baseline
Secondary	Proportion of renal crisis diagnosis in SSc patients		At baseline (Day 0) and 60 months after baseline
Secondary	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
Secondary	Mean of Diffusing capacity (DLCO) for the evaluation of disease activity for SSc patients		At baseline (Day 0) and 60 months after baseline
Secondary	Mean of Forced vital capacity (FVC) for the evaluation of disease activity for SSc patients		At baseline (Day 0) and 60 months after baseline
Secondary	Proportion of therapeutic strategies set up for SSc patients		At baseline (Day 0) and 60 months after baseline