Soluble CCN2/connective tissue growth factor levels in Egyptian systemic sclerosis patients: Possible association with cutaneous and pulmonary fibrosis

Abstract Aim of the work To assess plasma levels of CCN2 in systemic sclerosis (SSc) patients and study its relation with the disease characteristics. Patients and methods Plasma from 59 Egyptian patients with SSc (19 diffuse and 40 limited subtype) and 50 healthy controls were assayed for CCN2 levels by Enzyme-Linked Immunosorbent Assay (ELISA). Skin fibrosis was assessed using the modified Rodnan total skin thickness score (mRTSS). Results The mean age of patients was 39.4 ± 11.8 years (19–69 years), 41 females/18 males with mean disease duration of 6.6 ± 5.3 years. mRTSS was 13.1 ± 5.5 (median 12; range 3–28). Plasma CCN2 were significantly higher in patients with diffuse subtype (3296.2 ± 1540.5 pg/ml) than those with limited SSc (1984.4 ± 1174.1 pg/ml) (p = 0.001) and controls (1878.5 ± 501.2 pg/ml) (p = 0.78). In addition, plasma CCN-2 levels were increased in SSc patients with interstitial pulmonary fibrosis (2836.5 ± 1614.4 and 1861.6 ± 925.6 pg/ml, respectively; p = 0.04), in patients with early SSc than those with late (2843.1 ± 1586.7 and 2128.2 ± 1265.8 pg/ml respectively; p = 0.03). Patients with mRTSS ≥ 12 had higher CNN2 levels than those with lower scores (2783.6 ± 270.1 and 1842.9 ± 907.4 p = 0.03). CCN2 levels significantly correlated with mRTSS (r = 0.4, p = 0.002) and negatively with DLco (r = −0.39, P = 0.003) and FVC % (r = −0.38, r = 0.003). There was no significant relation to other organs involvement or to the presence of autoantibodies. Conclusion The work indicates the importance of CCN2 in cutaneous and pulmonary fibrosis in patients with SSc especially those with diffuse subtype. An additional prospective large scale, a longitudinal study should be carried out to support these findings and to reveal the mechanistic connections between CCN-2 levels and SSc disease manifestations.