Antiphospholipid syndrome

Antiphospholipid syndrome
Micrograph showing an advanced thrombotic microangiopathy, as may be seen in ALPA syndrome. Kidney biopsy. PAS stain.
Classification and external resources
Specialty	Rheumatology
ICD-10	D68.6 (ILDS D68.810)
ICD-9-CM	ICD9 289.81
OMIM	107320
DiseasesDB	775
eMedicine	med/2923
Patient UK	Antiphospholipid syndrome
MeSH	D016736
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Antiphospholipid syndrome or antiphospholipid antibody syndrome (APS or APLS), or often known also as Hughes syndrome in the UK, is an autoimmune, hypercoagulable state caused by antiphospholipid antibodies. APS provokes blood clots (thrombosis) in both arteries and veins as well as pregnancy-related complications such as miscarriage, stillbirth, preterm delivery, and severe preeclampsia.

The diagnostic criteria require one clinical event, i.e. thrombosis or pregnancy complication, and two antibody blood tests spaced at least three months apart that confirm the presence of either lupus anticoagulant, or anti-β₂-glycoprotein-I (since β₂-glycoprotein-I antibodies are a subset of anti-cardiolipin antibodies, an anti-cardiolipin assay can be performed as a less specific proxy).

Antiphospholipid syndrome can be primary or secondary. Primary antiphospholipid syndrome occurs in the absence of any other related disease. Secondary antiphospholipid syndrome occurs with other autoimmune diseases, such as systemic lupus erythematosus (SLE). In rare cases, APS leads to rapid organ failure due to generalised thrombosis; this is termed "catastrophic antiphospholipid syndrome" (CAPS or Asherson syndrome) and is associated with a high risk of death.

Antiphospholipid syndrome often requires treatment with anticoagulant medication such as heparin to reduce the risk of further episodes of thrombosis and improve the prognosis of pregnancy. Warfarin/Coumadin is not used during pregnancy because it can cross the placenta, unlike heparin, and is teratogenic.

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Wikipedia