Antiphospholipid Syndrome (APS)
Antiphospholipid syndrome (or antiphospholipid antibody syndrome) (APS) is a disorder of coagulation, which causes blood clots (thrombosis) in both arteries and veins, as well as pregnancy-related complications such as miscarriage, preterm delivery, or severe preeclampsia. The syndrome occurs due to the autoimmune production of antiphospholipid antibodies (aPL). The name Antiphospholipid Syndrome is a misnomer because the target antigen of aPL is not phospholipids but actually plasma proteins that bind to phospholipids (eg:] or prothrombin).
Antiphospholipid Syndrome is sometimes referred to as Hughes syndrome after the rheumatologist Dr. Graham R.V. Hughes (St. Thomas' Hospital, London, UK) who now works treating Lupus and Hughes Syndrome at the London Lupus Centre.
The term "Primary Antiphospholipid Syndrome" is used when APS occurs in the absence of any other autoimmune disease. APS is commonly seen in conjunction with other autoimmune diseases; the term "Secondary Antiphospholipid Syndrome" is used when APS coexists with other autoimmune diseases such as systemic lupus erythematosus (SLE). A very rare form of APS is the Catastrophic Antiphospholipid Syndrome, in which there is multiple and rapid organ thrombosis/dysfunction. The catastrophic APS carries a high mortality.