Open Access Open Badges Research article

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

Agneta Scheja1*, Anita Åkesson1, Pierre Geborek1, Marie Wildt1, Claes B Wollheim2, Frank A Wollheim1 and Ulrich M Vischer2

  • * Corresponding author: Agneta Scheja

Author Affiliations

1 Department of Rheumatology, University Hospital, Lund, Sweden

2 Division de Biochimie Clinique, Département de Médecine Interne, Centre Médical Universitaire, Geneva, Switzerland

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Arthritis Res 2001, 3:178-182  doi:10.1186/ar295

Published: 19 February 2001


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

systemic sclerosis; von Willebrand factor; von Willebrand factor propeptide