Antiphospholipid syndrome (APS) is an autoantibody-mediated acquired thrombophilia characterized by recurrent arterial or venous thrombosis and pregnancy morbidity. This V-LearningTM will help in understanding the antiphospholipid syndrome USMLE concepts.
Lecture Duration – 01:01:35
Release Date – January 2020
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Three major classifications of Antiphospholipid Antibodies are Antibodies against cardiolipin (aCL), Lupus anticoagulant (LA), and Antibodies against β2GPI (anti-β2GPI). APS may occur alone (primary) or in association with any other Systemic Autoimmune disease (secondary). Catastrophic APS (CAPS) is de ned as a rapidly progressive thromboembolic disease involving simultaneously three or more organs, organ systems, or tissues leading to corresponding functional defects.
Anti-PL plasma Protein Antibodies occur in 1–5% of the general population. One-third of patients with systemic lupus erythematosus possess these Antibodies, whereas their prevalence in other autoimmune connective tissue disorders, such as systemic sclerosis (scleroderma), Sjögren’s syndrome, dermatomyositis, Rheumatoid Arthritis, ranges from 6 to 15%. One-third of aPL-positive individuals experience thrombotic events or pregnancy morbidity.
Then comes the explanation of pathophysiology of antiphospholipid syndrome. Clinical manifestations represent mainly a direct or indirect expression of venous or arterial thrombosis and/or pregnancy morbidity. Clinical features associated with venous thrombosis are superficial and deep vein thrombosis, cerebral venous thrombosis, signs and symptoms of intracranial hypertension, retinal vein thrombosis, Pulmonary Emboli, pulmonary Arterial Hypertension, and Budd-Chiari syndrome. Pathogenesis of antiphospholipid syndrome is also explained here.
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The diagnosis of APS should be seriously considered in cases of thrombosis, cerebral vascular accidents in individuals younger than 55 years of age, or pregnancy morbidity in the presence of livedo reticularis or thrombocytopenia. Clinical criteria include vascular thrombosis and pregnancy morbidity.
Laboratory criteria include LA, anticardiolipin (aCL), and anti-β2GPI Antibodies. Differential diagnosis is based on the exclusion of other inherited or acquired causes o thrombophilia, Coombs-positive Hemolytic Anemia, and thrombocytopenia. Treatment of antiphospholipid syndrome icd 10 includes the after first thrombotic event and Recurrent Thrombotic Events.