Vasculopathy, Inflammation and Systemic Sclerosis

Study Overview
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Brief Summary
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It is a study of basic research with mechanistically objectives and including clinical biological samples.

Detailed Description
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Systemic sclerosis (SSc) is a rare and severe disease characterised by a fibrotic process and an incompletely elucidate physiopathology. Several shared featured have been identified between SSc and another autoimmune disease, the systemic lupus erythematous (SLE) as an interferon-alpha signature, the role of platelets and the polymorphism of OX40 ligand (OX40L). In SLE, OX40L has been shown highly linked to the active form of the disease, was increased by the CD40L of platelets and induced the CD8 cytotoxicity while inhibiting the suppressive functions of regulator T lymphocytes. The third main factor of the SSc physiopathology apart from autoimmunity and fibrosis is the vasculopathy with an important role of endothelial cells (EC). They turned out to be half-professional antigen presenting cells and can modulate the adaptive immunity.

Official Title
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Vasculopathy, Inflammation and Systemic Sclerosis: The Role of Endothelial Cell Activation and OX40/OX40L in Modulation of T Lymphocyte Activation

Conditions
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Systemic Sclerosis

Intervention / Treatment
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* Biological: Blood samples
* Biological: Biopsy


Participation Criteria
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Eligibility Criteria
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Inclusion Criteria: Age from 18 to 75. SSc diagnosed according to the American College of Rheumatology (ACR) criteria. With skin manifestations since less than 10 years. Localised sclerosis (LSc) diagnosed, morphea type. Exclusion Criteria: Age inferior to 18 or upper than 75. Skin manifestations since more than 10 years. Haemostasis diseases (independent from treatments). Stem cell transplant. Immunosuppressive treatments in the last 6 months. Associate autoimmune disease.

Ages Eligible for Study
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Minimum Age: 18 Years
Maximum Age: 75 Years

Sexes Eligible for Study
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All

Accepts Healthy Volunteers
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No

Study Plan
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How is the study designed?
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Design Details

Primary Purpose: Other
Allocation: Non-Randomized
Intervention Model: Parallel Assignment
Masking: None (Open Label)


Arms and Interventions

| Participant Group/Arm | Intervention/Treatment |
| --- | --- |
| Experimental: subjects SSc diagnosed<br> | Biological: Blood samples<br>* biological features of the standard follow-up 2 more blood tube for the biological collection (serum and PBMC)<br>Biological: Biopsy<br>* Skin biopsies<br>|
| Experimental: subjects Localised sclerosis diagnosed<br> | Biological: Blood samples<br>* biological features of the standard follow-up 2 more blood tube for the biological collection (serum and PBMC)<br>Biological: Biopsy<br>* Skin biopsies<br>|
| Experimental: subjects Sc<br> | Biological: Blood samples<br>* biological features of the standard follow-up 2 more blood tube for the biological collection (serum and PBMC)<br>|

What is the study measuring?
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Primary Outcome Measures

| Outcome Measure | Measure Description | Time Frame |
| --- | --- | --- |
| Assessment of OX40L expression in endothelial cells and skin biopsies. |  | Day 1 | 

 Terms related to the study
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Keywords Provided by Centre Hospitalier Valida
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T lymphocyte, OX40/OX40L