• Clinical science

Antiphospholipid syndrome

Summary

Antiphospholipid syndrome (APS) is an autoimmune disease that increases the risk of thrombosis as a result of procoagulatory antibodies. The condition may be idiopathic or acquired secondary to an underlying disease, such as systemic lupus erythematosus. Circulating antibodies deactivate anticoagulatory proteins and activate platelets, thereby inducing a hypercoagulable state. Typical clinical manifestations include recurring venous and arterial thrombotic events such as deep vein thromboses, strokes, or transient ischemic attacks. A severe complication of APS in women is recurrent miscarriages of healthy fetuses. The condition should be suspected in patients with a history of thrombosis or miscarriages. It is confirmed by detecting serum antiphospholipid antibodies (e.g., lupus anticoagulant, cardiolipin antibodies). Acute thrombotic events are treated with low-molecular-weight heparin and high-dose glucocorticoids to eliminate antibodies. In mild cases, long-term management involves prophylaxis with low dose aspirin; for patients at high risk of thrombotic events, warfarin is the drug of choice.

Etiology

Pathophysiology

Clinical features

APS usually manifests with recurring thrombotic events that may affect any organ.

Diagnostics

Treatment

The key element of APS treatment is systemic anticoagulation.

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  • 2. Miyakis S, Lockshin MD, Atsumi T, et al. International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS). J Thromb Haemost. 2006; 4(2): pp. 295–306. doi: 10.1111/j.1538-7836.2006.01753.x.
  • 3. Keeling D, Mackie I, Moore GW, Greer IA, Greaves M. Guidelines on the investigation and management of antiphospholipid syndrome. Br J Haematol. 2012; 157(1): pp. 47–58. doi: 10.1111/j.1365-2141.2012.09037.x.
last updated 10/22/2020
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