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

  1. Anonymous (2015), Antiphospholipid syndrome (English). Medical Subject Headings. U.S. National Library of Medicine.
  2. Levine JS, Branch DW, Rauch J (2002). "The antiphospholipid syndrome.". N Engl J Med 346 (10): 752-63. DOI:10.1056/NEJMra002974. PMID 11882732. Research Blogging.
  3. Vila P, Hernández MC, López-Fernández MF, Batlle J (1994). "Prevalence, follow-up and clinical significance of the anticardiolipin antibodies in normal subjects.". Thromb Haemost 72 (2): 209-13. PMID 7831653.
  4. Urbanus RT, Siegerink B, Roest M, Rosendaal FR, de Groot PG, Algra A (2009). "Antiphospholipid antibodies and risk of myocardial infarction and ischaemic stroke in young women in the RATIO study: a case-control study.". Lancet Neurol 8 (11): 998-1005. DOI:10.1016/S1474-4422(09)70239-X. PMID 19783216. Research Blogging.
  5. Ortona E, Capozzi A, Colasanti T, Conti F, Alessandri C, Longo A et al. (2010). "Vimentin/cardiolipin complex as a new antigenic target of the antiphospholipid syndrome.". Blood 116 (16): 2960-7. DOI:10.1182/blood-2010-04-279208. PMID 20634382. Research Blogging.
  6. 6.0 6.1 Miyakis S, Lockshin MD, Atsumi T, Branch DW, Brey RL, Cervera R et al. (2006). "International consensus statement on an update of the classification criteria for definite antiphospholipid syndrome (APS).". J Thromb Haemost 4 (2): 295-306. DOI:10.1111/j.1538-7836.2006.01753.x. PMID 16420554. Research Blogging.