Skip to main content
Log in

Fibrinolysis and coagulation abnormalities in systemic lupus erythematosus

Relationhip with Raynaud's phenomenon, disease activity, inflammatory indices, anticardiolipin antibodies and corticosteroid therapy

  • Original Article
  • Published:
Rheumatology International Aims and scope Submit manuscript

Abstract

Endothelial cell damage in systemic lupus erythematosus (SLE) was evaluated by measuring fibrinolytic activity and von Willebrand factor levels. Tissuetype plasminogen activator (t-PA) antigen, plasminogen activator inhibitor (PAI) activity, and von Willebrand factor antigen (vWF:Ag) and activity (vWF:RCof) were measured in 21 SLE patients (12 of whom were therapy free) and 22 controls. In addition, the relationship between such parameters and Raynaud's phenomenon, disease activity [according to personal criteria, Systemic Lupus Activity Measure (SLAM) and European Consensus Lupus Activity Measurement (ECLAM) scores], inflammatory indices [ESR, C-reactive protein (CRP), α2-globulin], anticardiolipin antibodies and corticosteroid therapy was investigated. Lower levels of t-PA antigen (P=0.003) and higher levels of vWF:Ag (P=0.001) were found in SLE patients in comparison with controls. Moreover, t-PA antigen was lower (P=0.02) in steroidfree patients in comparison with those taking steroids. No relationship was found between fibrinolysis and coagulation abnormalities and Raynaud's phenomenon, disease activity, inflammatory indices and anticardiolipin antibodies. Endothelial cell damage is probably a common feature in SLE patients; nervertheless, we were unable to clarify the nature of such abnormality. It is worth noting that low doses of steroids seem to be effective in improving endothelial cell function in SLE patients.

This is a preview of subscription content, log in via an institution to check access.

Access this article

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Similar content being viewed by others

References

  1. Kahaleh MB (1990) The role of vascular endothelium in the pathogenesis of connective tissue disease: endothelial injury, activation, participation and response. Clin Exp Rheumatol 8:595–601

    Google Scholar 

  2. Byron MA, Allington MJ, Chapel HM, Mowat AG, Cederholm-Williams SA (1987) Indications of vascular endothelial cell dysfunction in systemic lupus erythematosus. Ann Rheum Dis 46:741–745

    Google Scholar 

  3. Angles-Cano E, Sultan Y, Clauvel JP (1979) Predisposing factors to thrombosis in systemic lupus erythematosus. Possible relation to endothelial cell damage. J Lab Clin Med 94:312–323

    Google Scholar 

  4. Belch JJF, Zoma A, McLaughlin K, Curran L, Capell HA, Forbes CD, Sturrock RD (1987) Fibrinolysis in systemic lupus erythematosus: effect of desamino D-arginine vasopressin infusion. Br J Rheumatol 26:262–266

    Google Scholar 

  5. Jordan JM, Allen NB, Pizzo SV (1987) Defective release of tissue plasminogen activator in systemic and cutaneous vasculitis. Am J Med 82:397–400

    Google Scholar 

  6. Violi F, Ferro D, Valesini G, Quintarelli C, Saliola M, Grandilli MA, Balsano F (1990) Tissue plasminogen activator inhibitor in patients with systemic lupus erythematosus and thrombosis. BMJ 300:1099–1102

    Google Scholar 

  7. Woolf D, Wakerley G, Wallington TB, Scott DGI, Dieppe PA (1987) Factor VIII related antigen in the assessment of vasculitis. Ann Rheum Dis 46:441–447

    Google Scholar 

  8. Love PE, Santoro SA (1990) Ahtiphospholipid antibodies: anticardiolipin and lupus anticoagulant in systemic lupus erythematosus (SLE) and in non-SLE disorders. Prevalence and clinical significance. Ann Intern Med 112:682–698

    Google Scholar 

  9. Brey RL, Coull BM (1992) Antiphospholipid antibodies: origin, specificity, and mechanism of action. Stroke [Suppl I] 23:15–18

    Google Scholar 

  10. Ghirardello A, Doria A, Ruffatti A, Rigoli AM, Vesco P, Calligaro A, Gambari PF (1994) Antiphospholipid antibodies (aPL) in systemic lupus erythematosus (SLE). Are they specific tools for the diagnosis of aPL syndrome in SLE? Ann Rheum Dis 53:140–142

    Google Scholar 

  11. Tan EM, Cohen AS, Fries JF (1982) The 1982 revised criteria for the classification of systemic lupus erythematosus. Arthritis Rheum 25:1271–1277

    Google Scholar 

  12. Bencivelli W, Vitali C, Isenberg DA, Smolen JS, Snaith ML, Sciuto M, Bombardieri S and the European Consensus Study Group for Disease Activity in SLE (1992) Disease activity in systemic lupus erythematosus: report of the Consensus Study Group of the European Workshop for Rheumatology Research. III. Development of a computerised clinical chart and its application to the comparison of different indices of disease activity. Clin Exp Rheumatol 10:549–554

    Google Scholar 

  13. Liang MH, Socher SA, Larson MG, Schur PH (1989) Reliability and validity of six systems for the clinical assessment of disease activity in systemic lupus erythematosus. Arthritis Rheum 32:1107–1118

    Google Scholar 

  14. Laurell CB (1966) Quantitative estimation of proteins by electrophoresis in agarose gel containing antibodies. Anal Biochem 15:45–52

    Google Scholar 

  15. Casonato A, Fabris F, Vicariotto M, Girolami A (1985) The evaluation of factor VIII antigen by means of a simple slide test. Am J Clin Pathol 84:107–111

    Google Scholar 

  16. Thiagarajan P, Pengo V, Shapiro SS (1986) The use of the diluted Russell viper venom time for the diagnosis of lupus anticoagulants. Blood 4:869–874

    Google Scholar 

  17. Gharavi AE, Harris EN, Asherson RA, Hughes GRV (1987) Anticardiolipin antibodies: isotype distribution and phospholipid specificity. Ann Rheum Dis 46:1–6

    Google Scholar 

  18. Bunn CC, Gharavi AE, Hughes GRV (1982) Antibodies to extractable nuclear antigens in 173 patients with DNA-binding positive SLE: an association between antibodies to ribonucleoprotein and Sm antigens observed be counterimmuno-electrophoresis. J Clin Lab Immunol 8:13–17

    Google Scholar 

  19. Jaffe EA, Hoyer LW, Nachmann RL (1974) Synthesis of von Willebrand factor by cultured human endothelial cell. Proc Natl Acad Sci USA 71:1906–1911

    Google Scholar 

  20. Van Mourik JA, Lawrence DA, Loskutof DJ (1984) Purification of an inhibitor of plasminogen activator (anti-activator) synthesized by endothelial cells. J Biol Chem 259:14914–14921

    Google Scholar 

  21. Astedt B, Pandolfi M (1972) On release and synthesis of fibrinolytic activators in human organ culture. Eur J Clin Biol Res 17:261–267

    Google Scholar 

  22. Patrassi GM, Sartori MT, Casonato A, Viero ML, Pontara E, Saggiorato G, Girolami A (1992) Urokinase-type plasminogen activator release after DDAVP in von Willebrand disease: different behaviour of plasminogen activator according to the synthesis of von Willebrand factor. Thromb Res 66:517–526

    Google Scholar 

  23. Awada H, Barlowatz-Meimon G, Dourgados M, Maisonneuve P, Sultan Y, Amor B (1988) Fibrinolysis abnormalities in systemic lupus erythematosus and their relation to vasculitis. J Lab Clin Med 111:229–236

    Google Scholar 

  24. Nilsson TK, Löfvenberg E (1989) Decreased fibrinolytic capacity and increased von Willebrand factor levels as indicators of endothelial cell dysfunction in patients with lupus anticoagulant. Clin Rheumatol 8:58–63

    Google Scholar 

  25. Editorial (1993) Von Willebrand factor as a marker of injury to the endothelium in inflammatory vascular disease. J Rheumatol 20:1469–1471

    Google Scholar 

  26. Francis RB Jr (1989) Clinical disorders of fibrinolysis: a critical review. Blut 59:1–14

    Google Scholar 

  27. Blann AD, Illingworth K, Jayson MIV (1993) Mechanism of endothelial cell damage in systemic sclerosis and Raynaud's phenomenon. J Rheumatol 20:1325–1330

    Google Scholar 

  28. Jurado M, Paramo JA, Gutierrez-Pimentel M, Rocha E (1992) Fibrinolytic potential and antiphospholipid antibodies in systemic lupus erythematosus and other connective tissue disorders. Thromb Haemost 68:516–520

    Google Scholar 

  29. Ruiz-Argüelles GJ, Ruiz-Argüelles A, Lobato-Mednizábal E, Diaz-Gomez F, Pacheco-Pantoja E, Drenkard C, Alarcon-Segovia D (1991) Disturbances in the tissue plasminogen activator/plasminogen activator inhibitor (TPA/PAI) system in systemic lupus erythematosus. Am J Haematol 37:9–13

    Google Scholar 

  30. Wiman B, Hamsten A (1990) The fibrinolytic enzyme system and its role in the etiology of thromboembolic disease. Semin Thromb Hemost 16:207–216

    Google Scholar 

  31. Guariso G, Ruffatti A, Casonato A, Drigo P, Ghirardello A, Zancan L (1992) Antiphospholipid syndrome in a child with trisomy 21: the relationship between anticardiolipin G antibodies and the von Willebrand factor. Clin Exp Rheumatol 10:613–616

    Google Scholar 

  32. Rose PE, Struthers GS, Robertson M, Kavi J, Chant I, Taylor CM (1990) Factor VIII von Willebrand protein in haemolytic uraemic syndrome and systemic vasculitides. Lancet I: 500–502

    Google Scholar 

  33. Blann AD (1991) Von Willebrand factor antigen as an acute phase reactant and marker of endothelial cell injury in connective tissue disease: a comparison with CRP, rheumatoid factor, and erythrocyte sedimentation rate. Z Rheumatol 50:320–322

    Google Scholar 

  34. Isacson S (1970) Effect of prednisone on the coagulation and fibrinolytic systems. Scand J Haematol 7:212–216

    Google Scholar 

  35. Patrassi GM, Sartori MT, Viero ML, Scarano L, Boscaro M, Girolami A (1992) The fibrinolytic potential in patients with Cushing's disease: a clue to their hypercoagulable state. Blood Coag Fibrinol 3:789–793

    Google Scholar 

  36. Derksen RHWM, Hasselaar P, Oosting JD, De Groot PhG (1990) The antiphospholipid antibody dilemma. Clin Rheum 9 (S):39–44

    Google Scholar 

  37. Kordich LC, Forastiero RR, Basilotta E, Porterie P, Carreras LO (1992) Natural inhibitors of blood coagulation and fibrinolysis in patients with lupus anticoagulant. Blood Coagul Fibrinolysis 3:765–771

    Google Scholar 

  38. Matsuda J, Kawasugi K, Gohchi K, Saitoh N, Tsukamoto M, Kazama M, Kinoshita T (1992) Clinical significance of the venous occlusion test on systemic lupus erythematosus patients with a focus on changes in blood levels of tissue plaminogen activator, von Willebrand factor antigen, and thrombomodulin. Acta Haematol 88:22–26

    Google Scholar 

  39. Keeling DM, Campbell SJ, Mackie IJ, Machin SJ, Isenberg DA (1991) The fibrinolytic response to venous occlusion and the natural anticoagulants in patients with antiphospholipoid antibodies both with and without systemic lupus erythematosus. Br J Haematol 77:354–359

    Google Scholar 

  40. Francis RB, McGehee WG, Feinstein DI (1988) Endothelial-dependent fibrinolysis in subjects with the lupus anticoagulant and thrombosis. Thromb Haemost 59:412–414

    Google Scholar 

Download references

Author information

Authors and Affiliations

Authors

Rights and permissions

Reprints and permissions

About this article

Cite this article

Doria, A., Ghirardello, A., Boscaro, M. et al. Fibrinolysis and coagulation abnormalities in systemic lupus erythematosus. Rheumatol Int 14, 207–211 (1995). https://doi.org/10.1007/BF00262299

Download citation

  • Received:

  • Accepted:

  • Issue Date:

  • DOI: https://doi.org/10.1007/BF00262299

Key words

Navigation