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. 1994 Jan;70(1):40–43. doi: 10.1136/adc.70.1.40

von Willebrand factor antigen compared with other factors in vasculitic syndromes.

E Ateş 1, A Bakkaloğlu 1, U Saatçi 1, O Söylemezoğlu 1
PMCID: PMC1029680  PMID: 7906508

Abstract

In order to analyse their role as a specific marker of vascular damage and their value in monitoring disease activity the plasma concentrations of von Willebrand factor antigen (vWFAg) and the ristocetin cofactor (RiCoF) activities were determined in 43 children with vasculitis and 20 controls. These patients were sub-divided into three groups according to diagnosis: Henoch-Schönlein purpura (n = 18), polyarteritis nodosa (n = 16), and systemic lupus erythematosus (n = 9). High concentrations of vWFAg and activities of RiCoF were found in all the patient groups. vWFAg and RiCoF returned to normal as the patients became symptom free and remained above normal in those with continuing symptoms. The amount of vWFAg did not correlate with the acute phase reactants. vWFAg acted as a specific marker of vascular damage and was useful for the monitoring of disease activity both in small vessel vasculitis and systemic necrotising arteritis.

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Selected References

These references are in PubMed. This may not be the complete list of references from this article.

  1. Bowyer S. L., Ragsdale C. G., Sullivan D. B. Factor VIII related antigen and childhood rheumatic diseases. J Rheumatol. 1989 Aug;16(8):1093–1097. [PubMed] [Google Scholar]
  2. Cejka J. Enzyme immunoassay for factor VIII-related antigen. Clin Chem. 1982 Jun;28(6):1356–1358. [PubMed] [Google Scholar]
  3. Ewenstein B. M., Warhol M. J., Handin R. I., Pober J. S. Composition of the von Willebrand factor storage organelle (Weibel-Palade body) isolated from cultured human umbilical vein endothelial cells. J Cell Biol. 1987 May;104(5):1423–1433. doi: 10.1083/jcb.104.5.1423. [DOI] [PMC free article] [PubMed] [Google Scholar]
  4. Federici A. B., Fox R. I., Espinoza L. R., Zimmerman T. S. Elevation of von Willebrand factor is independent of erythrocyte sedimentation rate and persists after glucocorticoid treatment in giant cell arteritis. Arthritis Rheum. 1984 Sep;27(9):1046–1049. doi: 10.1002/art.1780270912. [DOI] [PubMed] [Google Scholar]
  5. Fretto L. J., Fowler W. E., McCaslin D. R., Erickson H. P., McKee P. A. Substructure of human von Willebrand factor. Proteolysis by V8 and characterization of two functional domains. J Biol Chem. 1986 Nov 25;261(33):15679–15689. [PubMed] [Google Scholar]
  6. Irazuzta J. E., Elbl F., Rees A. R. Factor VIII related antigen (von Willebrand's factor) in Kawasaki disease. Clin Pediatr (Phila) 1990 Jun;29(6):347–348. doi: 10.1177/000992289002900613. [DOI] [PubMed] [Google Scholar]
  7. Jaffe E. A., Hoyer L. W., Nachman R. L. Synthesis of von Willebrand factor by cultured human endothelial cells. Proc Natl Acad Sci U S A. 1974 May;71(5):1906–1909. doi: 10.1073/pnas.71.5.1906. [DOI] [PMC free article] [PubMed] [Google Scholar]
  8. Kahaleh M. B., Osborn I., LeRoy E. C. Increased factor VIII/von Willebrand factor antigen and von Willebrand factor activity in scleroderma and in Raynaud's phenomenon. Ann Intern Med. 1981 Apr;94(4 Pt 1):482–484. doi: 10.7326/0003-4819-94-4-482. [DOI] [PubMed] [Google Scholar]
  9. Lie J. T. Classification and immunodiagnosis of vasculitis: a new solution or promises unfulfilled? J Rheumatol. 1988;15(5):728–732. [PubMed] [Google Scholar]
  10. Lightfoot R. W., Jr, Michel B. A., Bloch D. A., Hunder G. G., Zvaifler N. J., McShane D. J., Arend W. P., Calabrese L. H., Leavitt R. Y., Lie J. T. The American College of Rheumatology 1990 criteria for the classification of polyarteritis nodosa. Arthritis Rheum. 1990 Aug;33(8):1088–1093. doi: 10.1002/art.1780330805. [DOI] [PubMed] [Google Scholar]
  11. Marder V. J., Mannucci P. M., Firkin B. G., Hoyer L. W., Meyer D. Standard nomenclature for factor VIII and von Willebrand factor: a recommendation by the International Committee on Thrombosis and Haemostasis. Thromb Haemost. 1985 Dec 17;54(4):871–872. [PubMed] [Google Scholar]
  12. Mills J. A., Michel B. A., Bloch D. A., Calabrese L. H., Hunder G. G., Arend W. P., Edworthy S. M., Fauci A. S., Leavitt R. Y., Lie J. T. The American College of Rheumatology 1990 criteria for the classification of Henoch-Schönlein purpura. Arthritis Rheum. 1990 Aug;33(8):1114–1121. doi: 10.1002/art.1780330809. [DOI] [PubMed] [Google Scholar]
  13. Nusinow S. R., Federici A. B., Zimmerman T. S., Curd J. G. Increased von Willebrand factor antigen in the plasma of patients with vasculitis. Arthritis Rheum. 1984 Dec;27(12):1405–1410. doi: 10.1002/art.1780271211. [DOI] [PubMed] [Google Scholar]
  14. Olson J. D., Brockway W. J., Fass D. N., Magnuson M. A., Bowie E. J. Evaluation of ristocetin-Willebrand factor assay and ristocetin-induced platelet aggregation. Am J Clin Pathol. 1975 Feb;63(2):210–218. doi: 10.1093/ajcp/63.2.210. [DOI] [PubMed] [Google Scholar]
  15. Rose P. E., Enayat S. M., Sunderland R., Short P. E., Williams C. E., Hill F. G. Abnormalities of factor VIII related protein multimers in the haemolytic uraemic syndrome. Arch Dis Child. 1984 Dec;59(12):1135–1140. doi: 10.1136/adc.59.12.1135. [DOI] [PMC free article] [PubMed] [Google Scholar]
  16. Sporn L. A., Chavin S. I., Marder V. J., Wagner D. D. Biosynthesis of von Willebrand protein by human megakaryocytes. J Clin Invest. 1985 Sep;76(3):1102–1106. doi: 10.1172/JCI112064. [DOI] [PMC free article] [PubMed] [Google Scholar]
  17. Tan E. M., Cohen A. S., Fries J. F., Masi A. T., McShane D. J., Rothfield N. F., Schaller J. G., Talal N., Winchester R. J. The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum. 1982 Nov;25(11):1271–1277. doi: 10.1002/art.1780251101. [DOI] [PubMed] [Google Scholar]
  18. Woolf A. D., Wakerley G., Wallington T. B., Scott D. G., Dieppe P. A. Factor VIII related antigen in the assessment of vasculitis. Ann Rheum Dis. 1987 Jun;46(6):441–447. doi: 10.1136/ard.46.6.441. [DOI] [PMC free article] [PubMed] [Google Scholar]

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