Antiphospholipid Syndrome

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  • Antiphospholipid Syndrome (APS) is an autoimmune disorder where the immune system mistakenly creates antibodies against certain phospholipids, which are normal components of cell membranes and blood proteins. 
  • These autoantibodies can cause blood clots to form in both arteries and veins, as well as cause pregnancy-related complications. The condition can occur on its own (primary APS) or in conjunction with other autoimmune disorders, most commonly systemic lupus erythematosus (secondary APS).
  • The blood clots that characterize APS can form in any blood vessel in the body, leading to a variety of symptoms depending on the location. When clots form in the legs, they can cause deep vein thrombosis (DVT); in the lungs, they can lead to pulmonary embolism; in the brain, they can cause stroke; and in the heart, they can trigger heart attacks. Some patients may also experience recurring miscarriages, particularly after the 10th week of pregnancy, as the antibodies can affect the placenta and interfere with normal pregnancy development.
  • Other common symptoms of APS include thrombocytopenia (low platelet count), livedo reticularis (a lace-like purple discoloration of the skin), headaches, cognitive difficulties, and heart valve abnormalities. In rare cases, patients may develop catastrophic antiphospholipid syndrome (CAPS), a life-threatening condition where blood clots form rapidly throughout multiple organs, leading to organ failure.
  • Diagnosis of APS requires both clinical criteria (thrombotic events or pregnancy complications) and laboratory testing. The main antibodies tested for include lupus anticoagulant, anticardiolipin antibodies, and anti-β2 glycoprotein I antibodies. These tests must be positive on two occasions at least 12 weeks apart to confirm the diagnosis, as these antibodies can temporarily appear during infections or with certain medications.
  • Treatment of APS primarily focuses on preventing blood clots through anticoagulation therapy. Most patients require lifelong treatment with blood thinners such as warfarin or heparin. During pregnancy, special management is required, typically involving a combination of heparin and low-dose aspirin to prevent both maternal thrombosis and pregnancy complications. Some patients may also need additional treatments, particularly if they have secondary APS associated with another autoimmune condition.
  • Prognosis varies among patients, but with proper treatment and monitoring, many people with APS can lead normal lives. Regular medical follow-up is essential to monitor anticoagulation levels and adjust treatment as needed. Patients must also be educated about the signs of blood clots and the importance of seeking immediate medical attention if these occur. Lifestyle modifications, such as avoiding prolonged immobility and maintaining a healthy weight, are also important aspects of managing the condition.
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