The authors submit a review on contemporary knowledge on potential candidates for markers of activity of systemic scleroderma. The submitted part I is focused on assessment of circulating levels of coagulation factors, markers of endothelial damage, vasoregulatory and growth factors and connective tissue components, their enzymes and inhibitors in different body fluids. A s to coagula - tion factors most knowledge has been assembled on the antigen of von Willebrand’s factor, plasminogen and thrombomodulin. As to vasoregulatory factors the greatest attention is still focused on changes of endothelin and recently nitric oxide. Propeptides of procollagen, posttranslation enzymes and proteinase inhibitors are most intensely studied markers of changes of connective tissue metabolism.

        Key words: systemic sclerosis, indicators of activity, coagulation factors, endothelin, co llagen