Increased von Willebrand factor antigen in the plasma of patients with vasculitis

Arthritis Rheum. 1984 Dec;27(12):1405-10. doi: 10.1002/art.1780271211.


The plasma concentrations of von Willebrand factor antigen (vWF:Ag) were determined in 101 patients who had the following diagnoses: vasculitis 8 patients, systemic lupus erythematosus (SLE) 51, rheumatoid arthritis (RA) 28, asthma 7, hereditary angioedema 7. The greatest mean concentration of vWF:Ag, 469% (normal 100% +/- 50), was observed in patients with vasculitis, often without elevation of the erythrocyte sedimentation rate. The mean concentration of vWF:Ag was also increased in both SLE (277%) and RA (194%). Twenty-four patients (15 with SLE, 6 with vasculitis, 3 with RA) had vWF:Ag concentrations greater than 300%. Four of these patients died within 1 year of the date of the study. Of the 15 SLE patients, 9 had vasculitis and 2 had active glomerulonephritis. The 3 RA patients had severe disease associated with extraarticular manifestations. Elevated vWF:Ag may reflect vascular damage, while markedly elevated levels of vWF:Ag appear to indicate a poor prognosis.

Publication types

  • Research Support, Non-U.S. Gov't
  • Research Support, U.S. Gov't, P.H.S.

MeSH terms

  • Angioedema / genetics
  • Angioedema / immunology
  • Antigens / analysis*
  • Arthritis, Rheumatoid / immunology
  • Asthma / immunology
  • Blood Sedimentation
  • Humans
  • Lupus Erythematosus, Systemic / immunology
  • Vasculitis / blood*
  • Vasculitis / immunology
  • von Willebrand Factor / immunology


  • Antigens
  • Von Willebrand antigen
  • von Willebrand Factor