Overview
Innate T cells (ITC) are decreased in systemic sclerosis (SS) and an early lymphocyte innateness has been reported. In the other part, ITC are implicated on inflammatory process, including the IL-33/ST2 axis, which is also involved in ScS endotheliopathy.
Data are however scarce and physiopathological mechanisms have not been assessed to date.
The investigators hypothesize a global lymphocyte innateness in SSc, linked to a chronic ITC stimulation by innate signals leading to ITC exhaustion, and their potential role in endotheliopathy and fibroblast activation in SSc.
Eligibility
Inclusion Criteria:
- SSc according to the 2013 ACR/EULAR 2013 criteria (or the 2001 Leroy's criteria for early SSc)
- Patients with others connective tissue disease:
- Systemic erythematosus lupus (SLE) according to the 2019 ACR/EULAR criteria
- Primary Sjögren syndrome (pSS) according to the 2016 ACR/EULAR criteria
- Rheumatoid arthritis according to the 2010 ACR/EULAR criteria
- Idiopathic inflammatory myopathy (IIM) according to the 2017 ACR/EULAR criteria
- Healthy subjects from general population without known autoimmune disease or
connective tissue disease
- ≥18 years-old
Exclusion Criteria:
- Overlap syndrome (including secondary Sjögren syndrome)
- Weight <55 kgs
- Known primary cell immunodeficiency
- Past of autologous or allogenic hematopoietic stem cell transplantation
- Solid neoplasia or malignant hemopathy in remission for less than 12 months an
- Chemotherapy and/or immune checkpoint inhibitors in the last 12 months
- Systemic retinoids
- Active infection and/or antibiotics in the last 2 weeks
- Known active chronic infection among HIV, HTLV, viral hepatitis, syphilis
- Vaccination in the last 4 weeks
- Subject refusing genetic analysis for the present study
- Pregnancy or breastfeeding