Seronegative Antiphospholipid Syndrome Associated with Plasminogen Activator Inhibitor

Abstract

Antiphospholipid syndrome is characterized by thrombosis, recurrent fetal loss, thrombocytopenia and is associated with the presence of antiphospholipid antibodies, especially anticardiolipin antibodies. We present a patient with the clinical features of antiphospholipid syndrome, namely recurrent venous and arterial thrombosis, recurrent abortions, thrombocytopenia and livido reticularis but with persistently negative serology for any type of antiphospholipid antibody. The possible existence of 'seronegative' antiphospholipid syndrome is proposed. Of additional interest in this patient is the presence of significantly elevated levels of plasminogen activator inhibitor. The role of this inhibitor, if any, in the antiphospholipid syndrome and/or seronegative antiphospholipid syndrome is not known and merits further study.

Keywords

Thrombosis Anticardiolipin antibody Plasminogen activator inhibitor Vasculopathy

Get full access to this article

View all access options for this article.

References

Gharavi AE , Harris EN , Asherson RA , Hughes Grv. Antiphospholipid antibodies: isotype distribution and phospholipid specificity . Ann Rheum Dis 1989; 46: 1-6.

Sabbaga J. , Neto JF , Chaddad R. , Cecconello I. , DeOliveira RM A 'primary' thrombotic syndrome: absence of anti-phospholipid antibodies . Clin Rheum 1991; 10: 81-3.

Hamsten A. , Defaire U. , Waldius G. et al. Plasminogen activator inhibitor in plasma: risk factor for recurrent myocardial infarction. Lancet 1987; ii: 3-9.

Hamsten A. , Wiman B. , deFaire U. , Blomback MN Increased plasma levels of a rapid inhibitor of tissue plasminogen activator in young survivors of myocardial infarction. New Engl J Med 1985; 313: 1557-63.

Schneiderman J. , Sawdey MS , Keeton MR et al. Increased type 1 plasminogen activator inhibitor gene expression in atherosclerotic human arteries. Proc Natl Acad Sci USA 1992; 89: 6998-7002.

Jorgensen M. , Philips M. , Thorsen S. , Selmer J. , Zeuthen J. Plasminogen activator inhibitor-1 is the primary inhibitor of tissue-type plasminogen activator in pregnancy plasma. Thromb Haemost 1987; 58: 872-8.

Paramo JA , Alfaro MJ , Rocha E. Postoperative changes in the plasmatic levels of tissue-type plasminogen activator and its fast-acting inhibitor relationship to deep vein thrombosis and influence of prophylaxis. Thromb Haemost 1985; 54: 713-16.

Suffredini AF , Harpel PC , Parillo JE Promotion and subsequent inhibition of plasminogen activation after administration of intravenous endotoxin to normal subjects . New Engl J Med 1989; 320: 1165-72.

Jurado M. , Paramo JA , Gutierrez-Pimentel M. , Rocha E. Fibrinolytic potential and antiphospholipid antibodies in SLE and other connective tissue disorders. Thromb Haemostas 1992; 68: 516-20.

10.

Violi F. , Ferro D. , Valesini G. et al. Tissue plasminogen activator inhibitor in patients with SLE and thrombosis. Br Med J 1990; 300: 1099-102.