Definition

Autoantibody-mediated acquired thrombophilia characterized by recurrent arterial or venous thromboses and/or pregnancy morbidity in the presence of autoantibodies against phospholipid (PL)-binding plasma proteins. Can occur alone (primary) or in association with another autoimmune disease (secondary).

Clinical Manifestations

Consist of vascular thrombotic features and pregnancy morbidity (Table 350-2, HPIM-20). Catastrophic APS (CAPS) is rapidly progressive thromboembolic disease involving three or more organ systems that can be life-threatening.

Evaluation

Laboratory examination of clotting parameters to include PTT, kaolin clotting time, dilute Russell viper venom test, antibodies directed against cardiolipin, β₂ glycoprotein, prothrombin. Antibodies should be measured on two occasions 12 weeks apart.

Diagnosis

Suggested by the presence of at least one clinical and one laboratory feature.