The HLA-B*35 allele modulates ER stress, inflammation and proliferation in PBMCs from Limited Cutaneous Systemic Sclerosis patients

The goal of this study was to assess whether the presence of HLA-B*35 contributes to activation of ER stress/UPR and inflammation in lcSScPAH PBMC. HLA-B*35 is associated with increased risk of developing PAH in lcSSc patietns. Also we previously reported that HLA-B*35 induces ECs dysfunction via activation of ER stress/UPR and upregulation of the inflammatory response. Our study demonstrates that HLA-B*35 contributes to the dysregulated expression of selected ER stress, inflammation and proliferation related genes in lcSSc patient PBMCs, as well as healthy individuals, thus supporting a pathogenic role of HLA-B*35 as a risk factor for developing PAH in SSc patients. In this study, PBMCs from 3 healthy controls were investigated.