With respect to SSc, increased CCN2 expression has been detected in patient groups, notably in blister fluids, sera, bronchoalveolar lavage fluids, skin and lung tissues, where CCN2 levels have been shown to correlate with clinical parameters, making CCN2 useful as a biomarker (Dziadzio et al., 2005; Hasegawa, 2016; Igarashi et al., 1995; Sato et al., 2000; Shi-wen et al., 2000). Here, CCN2 is linked to systemic sclerosis.