BLM is reported to induce pro-inflammatory cytokines such as TGFβ1 and CTGF in human dermal fibroblasts in vitro (43), suggesting that the combination of several pro-inflammatory mediators, even in the presence of TGFβ1, can increase the expression of MCHR1, consistent with our data in SSc dermal fibroblasts showing high levels of MCHR1. Here, CCN2 is linked to systemic sclerosis.