Antiphospholipid syndrome

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In medicine, antiphospholipid syndrome (APS) is the "presence of antibodies directed against phospholipids (antiphospholipid antibody). The condition is associated with a variety of diseases, notably systemic lupus erythematosus and other connective tissue diseases, thrombopenia, and arterial or venous thromboses. In pregnancy it can cause abortion. Of the phospholipids, the cardiolipins show markedly elevated levels of anticardiolipin antibodies (anticardiolipin antibody). Present also are high levels of lupus anticoagulant (lupus anticoagulant inhibitor)."[1][2]

Antibodies

The following antibodies are associated with the antiphopholipid syndrome; however, these antibodies may be present transiently in normal individuals.[3]

More recently identified antibodies are against the vimentin/cardiolipin complex.[5] Vimentina is a protein that binds to cardiolipin in vitro

Diagnosis

According to the Sapporo criteria, patients must have both:[6]

Clinical criteria

Laboratory evidence

Laboratory criteria according to an international consensus statement are:[6]
One of:

  • "Lupus anticoagulant present in plasma on two or more occasions at least 12 weeks apart."
  • "Medium or high titre (>40 IgG or IgM phospholipid units (1 unit is 1 µg of antibody), or >99th centile) of IgG or IgM anticardiolipin antibody in serum or plasma on two or more occasions, a least 12 weeks apart, measured by standardised enzyme linked immunosorbent assay (ELISA)."
  • "Medium or high titre (>40 IgG or IgM phospholipid units, or >99th centile) of IgG or IgM anti-ß2 glycoprotein I antibody in serum or plasma on two or more occasions, a least 12 weeks apart, measured by standardised ELISA, according to recommended procedures."

References