Primary antiphospholipid syndrome (PAPS) is an autoimmune disorder characterized by an antibody that binds to phospholipids, resulting in an increased risk of thrombosis and other clotting disorders. The exact cause of PAPS is unknown, but it is suspected to be related to genetic and environmental factors.
Diagnosis of PAPS is based on a combination of clinical, laboratory, and imaging findings. A positive antiphospholipid antibody test, such as lupus anticoagulant, anti-cardiolipin antibodies, or anti-beta-2-glycoprotein-I antibodies, is necessary for the diagnosis of PAPS. Other laboratory tests, such as complete blood count, prothrombin time, activated partial thromboplastin time, and platelet count may also be used to diagnose PAPS.
The differential diagnosis of PAPS includes other autoimmune disorders, such as systemic lupus erythematosus and thrombocytopenia. It is important to rule out these conditions before diagnosing PAPS.
Treatment of PAPS is aimed at preventing further thrombosis. This includes anticoagulant medications, such as warfarin and heparin, as well as antiplatelet medications, such as aspirin and clopidogrel. Other medications, such as steroids, immunosuppressants, and antimalarials, may also be used to reduce inflammation.
The prognosis of PAPS is generally good with proper treatment. People with PAPS are at an increased risk of recurrent thrombosis and other complications, but the risk is lower with appropriate treatment.