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  • Updated 05.04.2021
  • Released 01.02.2008
  • Expires For CME 05.04.2024

Antiphospholipid antibody syndrome

Introduction

Overview

Antiphospholipid syndrome is a thrombophilic disorder that involves multiple organ systems. It should always be suspected in younger patients, especially obstetric patients presenting with thromboembolic conditions, including strokes. A fulminant form of disease can 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 disorder characterized by a hypercoagulable state and recurrent miscarriages in presence of persistent antiphospholipid antibodies.

• Cerebral arteries are a common site of arterial thrombosis due to antiphospholipid antibody syndrome.

• Screening for antiphospholipid antibodies is recommended for young patients presenting with cryptogenic ischemic strokes.

• 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

Extensive screening for syphilis in the 1950s led to the finding that some individuals had false positive tests. These results were often associated with other infections (Moore and Mohr 1952). Furthermore, some of these individuals later developed systemic lupus erythematosus (67). Patients with systemic lupus erythematosus had circulating antibodies associated with thromboembolism (14). These antibodies were named “lupus anticoagulant” because they were associated with elevated activated partial thromboplastin time (aPTT) in vitro (39). Later, lupus anticoagulant was linked to recurrent abortions (69).

In the 1980s, antiphospholipid syndrome was described as the association of anticardiolipin antibodies with arterial and/or venous thrombosis (51). Anticardiolipin bind to cardiolipin only in the presence of β2-glycoprotein 1 (β2GPI), an inhibitor of platelet activation and coagulation (45; 63). Anticardiolipin antibodies from patients with systemic lupus erythematous, but not from patients with infections, required β2GPI in order to bind to cardiolipin (52). Currently, lupus anticoagulant, anticardiolipin, and anti-β2GPI are all considered to be pathogenic in the clinical spectrum of antiphospholipid syndrome.

Antiphospholipid syndrome is a clinical syndrome 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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