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Diagnosis of antiphospholipid syndrome

Doruk Erkan, MD, MPH
Thomas L Ortel, MD, PhD
Section Editor
David S Pisetsky, MD, PhD
Deputy Editors
Monica Ramirez Curtis, MD, MPH
Jennifer S Tirnauer, MD


Antiphospholipid syndrome (APS) is characterized by venous or arterial thrombosis and/or an adverse pregnancy outcome in the presence of persistent laboratory evidence of antiphospholipid antibodies (aPL). APS occurs either as a primary condition or in the setting of an underlying disease, usually systemic lupus erythematosus (SLE).

The diagnosis of APS will be reviewed here. The clinical manifestations and treatment of this disorder are presented separately. (See "Pathogenesis of antiphospholipid syndrome" and "Clinical manifestations of antiphospholipid syndrome" and "Treatment of antiphospholipid syndrome".)

The effect of aPL on coagulation tests is also discussed separately. (See "Clinical use of coagulation tests".)


Antiphospholipid syndrome – Antiphospholipid syndrome (APS) is a systemic autoimmune disorder characterized by venous or arterial thrombosis and/or pregnancy morbidity in the presence of persistent laboratory evidence of antiphospholipid antibodies (aPL) [1]. APS occurs as a primary condition, or it can occur in the presence of systemic lupus erythematosus (SLE) or another systemic autoimmune disease.

Antiphospholipid antibodies – aPL are a heterogeneous group of antibodies directed against phospholipid-binding proteins [2]. The aPL included in international classification criteria are anticardiolipin (aCL) antibody (immunoglobulin G [IgG] or IgM), anti-beta2-glycoprotein (GP) I antibody (IgG or IgM), and lupus anticoagulant (LA) (see 'Classification criteria' below). Although cardiolipin is a phospholipid, most of the clinically relevant antibodies detected in this assay are actually binding to phospholipid-binding protein(s), frequently beta2-GP I, that bind to the cardiolipin in the assay. There are other aPL that are not included in international classification criteria (eg, antibodies directed against prothrombin, phosphatidylserine, or phosphatidylinositol) which are not routinely obtained because of lack of standardized testing and uncertainty about their clinical significance. (See 'Antiphospholipid antibody testing' below.)


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Literature review current through: Sep 2016. | This topic last updated: Sep 30, 2016.
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