The antiphospholipid syndrome (APS) is a noninflammatory autoimmune disease defined by the presence of antiphospholipid antibodies APA in the plasma of patients with venous or arterial thrombosis, or complications of pregnancy (Table 63.1). APS occurs predominantly in young women and is characterized by recurrence and high morbidity.
Primary antiphospholipid syndrome refers to patients with the syndrome who do not have any other rheumatological or autoimmune conditions such as lupus erythematosus. Associated (but not defining) conditions include thrombocytopenia, vasculitic rashes, arthralgias, dermal necrosis of digits, livedo reticularis, and pulmonary hypertension. A thrombotic cause of these additional manifestations is unlikely since anticoagulant treatment does not result in a remission of these complaints.
The presence of APL antibodies can be detected by either a phospholipid-dependent prolongation of a coagulation test [lupus anticoagulant (LAC)] or as anticardiolipin antibodies (ACA) in a b2-glycoprotein I (b2-GPI)-dependent enzyme-linked immunosorbent assay (ELISA). LAC activity and ACA are closely related but not identical. Patients should be tested with both types of assays (immunologic and clot-based) to rule out the diagnosis of antiphospholipid syndrome in a patient with a clinical presentation consistent with this disorder.
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