Antiphospholipid Syndrome Overview

Antiphospholipid Syndrome Overview

• An autoimmune disorder characterized by the presence of antiphospholipid antibodies that target cell surface proteins linked to phospholipids.
• Leads to inflammation and heightened risk of thrombosis (formation of blood clots).
• Can occur alone or in conjunction with other autoimmune diseases, especially systemic lupus erythematosus (SLE).

Antiphospholipid Antibodies

• Lupus Anticoagulant: Impairs the body's ability to regulate clotting processes.
• Anticardiolipin Antibodies: Associated with increased clotting risk, found in many patients with antiphospholipid syndrome.
• Anti-beta-2 Glycoprotein I Antibodies: Involved in pathogenesis and associated with thrombosis.

Key Complications

• Venous Thromboembolism: Includes deep vein thrombosis and pulmonary embolism as major risks.
• Arterial Thrombosis: Can lead to serious conditions like stroke, myocardial infarction, and renal thrombosis.
• Pregnancy-Related Complications: Associated risks include recurrent miscarriages, stillbirths, and pre-eclampsia.

Catastrophic Antiphospholipid Syndrome

• A rare but severe variant marked by rapid thrombosis across multiple organs within days, leading to high mortality.
• Important to diagnose in patients with thrombosis history and recurrent miscarriage.

Associated Symptoms and Conditions

• Livedo Reticularis: A mottled, lace-like purple rash linked to the syndrome, with a more persistent form known as livedo racemosa.
• Libmann-Sacks Endocarditis: Non-bacterial endocarditis characterized by vegetations on heart valves, notably in mitral and aortic positions.
• Thrombocytopenia: Low platelet count is frequently observed in patients.

Diagnosis and Management

• Diagnosis relies on clinical presentation and detection of persistent antiphospholipid antibodies.
• Long-Term Management: Warfarin prescribed with a target INR of 2-3 to minimize thrombosis risk.
• Pregnancy Management: Low molecular weight heparin (e.g. enoxaparin) and aspirin are utilized to lower thrombosis risks during pregnancy; Warfarin is contraindicated due to risks to the fetus.

This quiz covers the key aspects of Antiphospholipid Syndrome, including its autoimmune nature, associated antibodies, and implications for health. Learn about how these antibodies interact with phospholipids, leading to inflammation and thrombosis risk. Ideal for those studying immunology and related medical fields.