Von Willebrand factor propeptide as a marker of disease activity in systemic sclerosis (scleroderma)

In 44 consecutive patients with systemic sclerosis (SSc), plasma concentrat ions of von Willebrand factor (vWf) were higher than those of the vWf prope ptide, but the propeptide showed less variability within patient subgroups. Higher values of the propeptide were observed in patients with early pulmo nary involvement. A closer correlation of the propeptide than of vWf to bio chemical markers of activity was also evident. Our results suggest that the propeptide, despite a shorter circulating half-time and lower plasma conce ntrations than vWf, is more useful in the assessment of disease activity in SSc.