The Open Autoimmunity Journal

2010, 2 : 67-75
Published online 2010 March 11. DOI: 10.2174/1876894601002010067
Publisher ID: TOAUTOJ-2-67

Clinical Management of Antiphospholipid Syndrome-Related Thrombosis

Gerard Espinosa and Ricard Cervera
Department of Autoimmune Diseases, Institut Clínic de Medicina i Dermatologia, Hospital Clínic, Barcelona, Catalonia, Spain

ABSTRACT

There is evidence that the presence of antiphospholipid antibodies is related with an increased risk of thrombotic events. Patients with definite antiphospholipid syndrome (APS) and a first venous event should receive long term oral anticoagulation to an international normalized ratio (INR) of 2.0-3.0. In patients with definite APS and arterial or recurrent thrombosis oral anticoagulation to an INR > 3.0 may be advisable. Catastrophic antiphospholipid syndrome is an unusual form of presentation of antiphospholipid syndrome with a mortality rate of approximately 50%. Its treatment is based on the combined use of full anticoagulation, corticosteroids, plasma exchanges, and intravenous immunoglobulins. We also summarize the evidence-based information about management of some difficult cases such as “seronegative” APS and patients who do not display formal classification criteria for APS.

Keywords:

Antiphospholipid syndrome, thrombosis, oral anticoagulation, catastrophic antiphospholipid syndrome.