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Updated: Jun 12, 2026

Imaging Features of Systemic Sclerosis-Associated Interstitial Lung Disease
Published on: June 16, 2020
[Systemic sclerosis and scleroderma circumscripta--disturbances of selected serum parameters which are responsible
Bozena Dziankowska-Bartkowiak1, Agnieszka Zebrowska, Małgorzata Wagrowska-Danielewicz
1Zakład Immunodermatologii, Kliniki Dermatologii UM w Łodzi. bozenadz@interia.pl
Abstract:
Scleroderma is a connective tissue disease characterized by fibrosis confined only to skin (scleroderma circumscripta, morphea) or to skin and internal organs (systemic sclerosis, SSc) as a result of vascular changes, immune dysfunction and increased production of collagen and other extracellular matrix (ECM) components. Both types of scleroderma present clinical and histological similarities in skin changes but their pathogenic relationship is still not elucited. The aim of our study was to evaluate vascular changes in both types of scleroderma on the basis of: serum levels of gelatinases--MMP-2 and MMP-9, vascular endothelial growth factor (VEGF) and its soluble receptor 2 (sVEGFR2) and expression of CD34 antigen in skin changed samples. The following patients were involved in the study: 34 patients with SSc, 31 with morphea and 15 healthy controls. Serum levels of selected parameters were estimated by ELISA method; whereas CD34 antigen immunoexpression was performed by immunohistochemistry. Serum level of MMP-9 was statistically significantly higher in SSc and morphea patients compared to the control group (848.6 ng/ml; 844.4 ng/ml vs. 535.9 ng/ml; p<0.05). Statistical analysis revealed that mean serum levels of MMP-2 was significantly (p<0.05) higher in SSc group compared to morphea and control group (240.4 ng/ml vs. 208.1 ng/ml; 211.9 ng/ml) and VEGF was higher in morphea group compared to the control group (422.2 pg/ml vs. 188.7 pg/ml). Statistically significant difference between ratio VEGF/sVEGFR2 of mean serum levels in SSc patients and the control group (0.038 pg/ml vs. 0.018 pg/ml) and positive correlation between serum levels of MMP-9 and sVEGFR2 only in control group (r=0.143) were obtained. Possitive CD34 expression was found in vascular endothelial cells cytoplasm. Mean value of immunoexpression of CD34 was statistically significantly higher in the control group (2.8 +/- 0.42) compared to SSc group (1.59 +/- 0.69) and morphea (1.42 +/- 0.50) (p<0.001). There was no statistically significant difference between immunoexpression of CD34 in skin samples of both types of scleroderma (p=0.27). The obtained results seem to confirm pathogenic similarities in endothelial cells disturbances in both types of scleroderma--SSc and morphea.
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