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  • Updated 06.30.2022
  • Released 01.02.2008
  • Expires For CME 06.30.2025

Antiphospholipid antibody syndrome

Introduction

Overview

Antiphospholipid syndrome is a coagulopathy affecting multiple organ systems. It should be suspected in young patients, presenting with unexplained venous thrombosis, arterial infarcts, and/or miscarriages. A fulminant form of widespread coagulopathy may complicate some cases. The cornerstone of therapy is anticoagulation. In this article, the authors provide a comprehensive review of the disorder, including clinical manifestations, pathogenesis, and management.

Key points

• Antiphospholipid antibody syndrome is a hypercoagulable state associated with persistent antiphospholipid antibodies.

• Cerebral arteries are a common site of thrombosis.

• Screening for antiphospholipid antibodies is recommended in young patients presenting with unexplained ischemic strokes or recurrent miscarriages.

• Catastrophic antiphospholipid syndrome is a rare, but life threatening, presentation with simultaneous involvement of multiple organ systems.

• Antiplatelets and anticoagulation are the mainstay treatments for thrombotic complications, with a potential role of various immunosuppressants in some complicated cases.

Historical note and terminology

Screening for syphilis has revealed false positive tests often associated with other infections (Moore and Mohr 1952). Some of the individuals later developed systemic lupus erythematosus (73). Moreover, patients with systemic lupus erythematosus had circulating antibodies associated with thromboembolism (15). These antibodies were named “lupus anticoagulant” because of the associated elevation of activated partial thromboplastin time (aPTT) (41). Later, lupus anticoagulant was linked to recurrent abortion (75).

Thrombosis associated with anticardiolipin antibodies was described as antiphospholipid syndrome (55). Anticardiolipin antibodies bind to cardiolipin only in the presence of β2-glycoprotein 1 (β2GPI), an inhibitor of platelet activation and coagulation (47; 68). Anticardiolipin antibodies from patients with systemic lupus erythematous, but not from patients with infections, require β2GPI to bind to cardiolipin (56). Currently, lupus anticoagulant, anticardiolipin, and anti-β2GPI are all considered to be pathogenic within the clinical spectrum of antiphospholipid syndrome.

Antiphospholipid syndrome is associated with recurrent arterial or venous thrombosis, spontaneous abortion, and thrombocytopenia in patients with moderate to high titers of antiphospholipid measured twice at 12 weeks apart. It can be divided into 2 subtypes:

• Primary antiphospholipid syndrome in the absence of autoimmune disorders
• Secondary antiphospholipid syndrome associated with autoimmune disorders

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