Antiphospholipid syndrome (APS)
Definition-Antiphospholipid syndrome (APS)
Features:
DVT/PE/CVA, recurrent fetal loss, ↓ plts, hemolytic anemia, livedo reticularis
“Catastrophic APS”:
≥3 organ systems in <1 wk w/ ⊕ APLA & tissue microthrombi; 44% mortality (Arth Rheum 2006;54:2568); Rx w/ plasmapheresis, rituximab
This is a description of the clinical features and treatment of a severe autoimmune disease called antiphospholipid antibody syndrome (APLA). APLA is characterized by the formation of antibodies against phospholipids, which are crucial components of cell membranes. These antibodies can cause a wide range of problems, including thrombosis (blood clots), stroke, and organ damage. APLA is a very serious condition, with a mortality rate of 44%. Treatment typically involves plasmapheresis (removal of antibodies from the blood) and rituximab (a drug that suppresses the immune system)
Antiphospholipid antibodies (APLA)
Etiologies:
- primary (idiopathic) or secondary due to autoimmune syndromes (eg, SLE)
- malignancy
- infections
- drug reactions