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  • Updated 08.25.2019
  • Released 01.02.2008
  • Expires For CME 08.25.2022

Antiphospholipid antibody syndrome

Introduction

This article includes discussion of antiphospholipid antibody syndrome, APS, and Hughes syndrome. The foregoing terms may include synonyms, similar disorders, variations in usage, and abbreviations.

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.

• Cerebral arteries are a common site of arterial thrombosis secondary 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 of the patients with positive venereal disease research laboratory tests had no clinical features of the disease. Moore and Mohr described this subpopulation of individuals and found that these false positive results were often associated with other infectious diseases (Moore and Mohr 1952). Furthermore, when followed for certain period of time, some of these individuals were at higher risk for developing systemic lupus erythematosus (69). Bowie and colleagues demonstrated that patients with systemic lupus erythematosus had circulating antibodies, which predisposed them to thromboembolic events (15). Feinstein and Rapaport renamed these antibodies “lupus anticoagulant” (LAC) because they were associated with elevated activated partial thromboplastin time (aPTT) (40). In 1975, Nilsson and colleagues identified LAC as also being associated with recurrent abortions (71).

In the 1980s, Hughes and colleagues did pioneering work to separate a distinct disease entity that was associated with anticardiolipin (aCL) antibodies and increased arterial as well as venous thrombosis (52), which was named “antiphospholipid syndrome.” Continued work on these antibodies revealed that some of these antiphospholipid antibodies required the presence of β2-glycoprotein, a plasma phospholipid-binding protein for binding cardiolipin (46; 65). This was exclusive to patients with systemic lupus erythematous and antiphospholipid syndrome, but not exhibited by syphilitic patients (46; 65; 53). This led to the discovery of anti-β2-glycoprotein I (anti-β2GpI) antibodies as separate from LAC as a separate entity (46; 03). Currently, presence of LAC, aCL, 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 circulating antiphospholipid antibodies. It can be divided into two subtypes:

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

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