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

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

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.

Study Overview

• Status: Recruiting
• Conditions: Systemic Sclerosis; Scleroderma
• Intervention / Treatment: Biological: Blood samples; Other: Biopsy; Other: Bronchoalveolar samples

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.

• Study Type: Interventional
• Enrollment (Estimated): 500
• Phase: Not Applicable

Contacts and Locations

• Study Contact: Name: Marie-Elise TRUCHETET, MD, PhD; Phone Number: +33 05.56.79.55.56; Email: marie-elise.truchetet@chu-bordeaux.fr
• Study Contact Backup: Name: Thomas BARNETCHE, PhD; Phone Number: +33 05.57.82.04.93; Email: thomas.barnetche@chu-bordeaux.fr

Study Locations

• France
  • Bordeaux, France
    • Recruiting
    • CHU de Bordeaux - service de rhumatologie
    • Sub-Investigator: Joel CONSTANS, Prof
    • Contact: Marie-Elise TRUCHETET, MD, PhD; Phone Number: +33 05.56.79.55.56; Email: marie-elise.truchetet@chu-bordeaux.fr
    • Contact: Thomas BARNETCHE, PhD; Phone Number: +33 05.57.82.04.93; Email: thomas.barnetche@chu-bordeaux.fr
    • Sub-Investigator: Elodie BLANCHARD, MD
    • Sub-Investigator: Francois PICARD, MD
    • Principal Investigator: Marie-Elise Truchetet, MD
    • Sub-Investigator: Estibaliz LAZARO, Prof
    • Sub-Investigator: Julien SENESCHAL, Prof
    • Sub-Investigator: Pierre DUFFAU, Prof

Participation Criteria

Eligibility Criteria

• Ages Eligible for Study: 18 years and older (Adult, Older Adult)
• Accepts Healthy Volunteers: No

Description

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

Study Plan

How is the study designed?

Design Details

• Primary Purpose: Other
• Allocation: N/A
• Interventional Model: Single Group Assignment
• Masking: None (Open Label)

Number of Arms

1

Arms and Interventions

Participant Group / Arm	Intervention / Treatment
Experimental: subjects SSc diagnosed; Patient with systemic scleroderma according to the American College of Rheumatology (ACR) / EULAR 2013 criteria	Biological: Blood samples; 62 ml whole blood for Peripheral blood mononuclear cell (PBMC) and monocytes isolation; Other: Biopsy; Skin biopsies; Other: Bronchoalveolar samples; 50 ml of bronchoalveolar samples if pulmonary flare requires this type of exploration

What is the study measuring?

Primary Outcome Measures

Outcome Measure	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

Secondary Outcome Measures

Outcome Measure	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
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

Collaborators and Investigators

• Sponsor: University Hospital, Bordeaux
• Investigators: Principal Investigator: Marie-Elise TRUCHETET, MD, PhD, University Hospital, Bordeaux

Study record dates

Study Major Dates

• Study Start (Actual): June 8, 2020
• Primary Completion (Estimated): June 1, 2030
• Study Completion (Estimated): June 1, 2030

Study Registration Dates

• First Submitted: February 5, 2020
• First Submitted That Met QC Criteria: February 10, 2020
• First Posted (Actual): February 11, 2020

Study Record Updates

• Last Update Posted (Estimated): January 19, 2024
• Last Update Submitted That Met QC Criteria: January 18, 2024
• Last Verified: January 1, 2024

More Information

Terms related to this study

• Keywords: Cohort study; prognosis; systemic sclerosis
• Additional Relevant MeSH Terms: Pathologic Processes; Skin Diseases; Connective Tissue Diseases; Sclerosis; Scleroderma, Systemic; Scleroderma, Diffuse
• Other Study ID Numbers: CHUBX 2019/42

Drug and device information, study documents

• Studies a U.S. FDA-regulated drug product: No
• Studies a U.S. FDA-regulated device product: No