Increased von Willebrand factor antigen in the plasma of patients with vasculitis.
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.
D000799:Angioedema / Q000235:genetics / Q000276:immunology; D000941:Antigens / Q000032:analysis*; D001172:Arthritis, Rheumatoid / Q000276:immunology; D001249:Asthma / Q000276:immunology; D001799:Blood Sedimentation; D006801:Humans; D008180:Lupus Erythematosus, Systemic / Q000276:immunology; D014657:Vasculitis / Q000097:blood* / Q000276:immunology; D014841:von Willebrand Factor / Q000276:immunology
D000941:Antigens; C029417:Von Willebrand antigen; D014841:von Willebrand Factor