Systemic scleroderma (SSc) is a disease characterized by vascular damage, skin and internal organ connective tissue thickening or fibrosis. Variability of clinical symptoms, chronic course of the disease and little value of acute-phase reactant measurement, all contribute to the lack of specific markers for the disease activity, which would help with indication of treatment and further follow up of SSc. The aim of this study was to verify if some selected factors could serve as SSc activity markers. Clinical correlations of potential markers of disease activity, determined in a group of SSc patients at the start of the study and after 1 year were compared. In total 49 patients were examined – 36 with limited, 9 with diffuse and 4 with other forms of SSc. The circulating levels of N-terminal propeptide of procollagen type III (PNPIIIP), interleukin-6 (IL-6), soluble interleukin-2 receptor (sIL2-R), intercellular adhesion molecule-1 (sICAM-1), vascular adhesion molecule (sVCAM-1), of von Willebrands factor antigen (vWFAg), big endothelin-1 (BET-1), and urine excretion of pyridinoline (PYR)anddeoxypyridinoline (D-PYR)were determined.Then the correlations of these markers with clinical data reflecting activity and with functional questionnaire (FQ) were measured. The mean levels ofsICAM-1,sVCAM-1,vWFAg,sIL-2R,BET-1 andPYRwerenot significantly elevatedcompared with normal levels. Concentration of NPIIIP, D-PYR and IL-6 were normal. No significant difference was found in the levels of any of these markers at control measurement after 1 year. The level of NPIIIP correlated with the finger to palm (FTP) distance as well as with D-PYR concentration and FQ. IL-6 levels correlated with leukocyte count, sIL-2R and FQ. The same correlations were found for NPIIIP and D-PYR after 1 year. IL-6 levels and FQ, even sIL-2Rand erythrocyte count correlated. BET-1 concentration and decreased DLCO correlated. Correlations of collagen turnover markers with skin damage indicators and with FQ, conforming with literature data, support their possible use for monitoring of fibrotic changes. Correlation of endothelial markers damage with activation of the immune system will have to be verified in larger studies.

        Key words: systemic sclerosis, activity, collagen, adhesion molecules, interleukins, endothelin