VON-WILLEBRAND-FACTOR - INCREASED LEVELS ARE RELATED TO POOR-PROGNOSIS IN SYSTEMIC-SCLEROSIS AND NOT TO TISSUE AUTOANTIBODIES

von Willebrand factor, C-reactive protein, rheumatoid factor, major or gan involvement, antibodies to extractable nuclear antigen, anti-cardi olipin antibodies, anti-centromere antibodies and anti-nuclear antibod ies were measured in 33 patients with systemic sclerosis. After five y ears, the nine patients who had died had initial levels of von Willebr and factor significantly higher (median 288 IU/dL, range 150-1170) tha n levels in the 24 who were still alive (median 148 IU/dL, range 65-26 2, Mann-Whitney P=0.0002). Increased levels of von Willebrand factor c orrelated with the time interval from blood sampling until the patient 's death (Spearman's r=0.73, P=0.02). Levels of C-reactive protein, rh eumatoid factor, and tissue autoantibodies, and age or the number of o rgans involved were unable to predict this outcome. We suggest that en dothelial perturbation, as indicated bq: increased levels of von Wille brand factor, is a strong indicator of a poor prognosis in systemic sc lerosis.