title: von Willebrand factor: increased levels are related to poor prognosis in systemic sclerosis and not to tissue autoantibodies.
abstract: von Willebrand factor, C-reactive protein, rheumatoid factor, major organ involvement, antibodies to extractable nuclear antigen, anti-cardiolipin antibodies, anti-centromere antibodies and anti-nuclear antibodies were measured in 33 patients with systemic sclerosis. After five years, the nine patients who had died had initial levels of von Willebrand factor significantly higher (median 288 IU/dL, range 150-1170) than levels in the 24 who were still alive (median 148 IU/dL, range 65-262, Mann Whitney P = 0.0002). Increased levels of von Willebrand factor correlated 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, rheumatoid factor, and tissue autoantibodies, and age or the number of organs involved were unable to predict this outcome. We suggest that endothelial perturbation, as indicated by increased levels of von Willebrand factor, is a strong indicator of a poor prognosis in systemic sclerosis.
authors.affiliation: Department of Rheumatology, The Medical School, University of Birmingham, England, UK.
authors.forename: A D
authors.initials: AD
authors.lastname: Blann
authors.forename: T P
authors.initials: TP
authors.lastname: Sheeran
authors.forename: P
authors.initials: P
authors.lastname: Emery
mesh_terms: D000328:Adult; D000368:Aged; D001323:Autoantibodies / Q000378:metabolism*; D005260:Female; D005500:Follow-Up Studies; D006801:Humans; D008297:Male; D008875:Middle Aged; D011379:Prognosis; D011446:Prospective Studies; D012595:Scleroderma, Systemic / Q000097:blood* / Q000276:immunology; D014841:von Willebrand Factor / Q000378:metabolism*
chemical_list: D001323:Autoantibodies; D014841:von Willebrand Factor