Antiphospholipid antibody syndrome

Antiphospholipid antibody syndrome (APS) is an autoimmune disease characterised by a combination of vascular thrombosis and/or pregnancy-related complications and the long-term presence of antibodies directed against phospholipids, i.e. circulating anticoagulant and/or anticardiolipin antibodies and/or antiβ2 glycoprotein 1 antibodies. The disease may occur alone (primary APS) or combined with another disease (secondary APS), i.e. generally systemic lupus erythematosus. Thrombosis most often affects the veins of the legs (phlebitis) and the arteries of the brain (stroke). The most common dermatologic manifestation is livedo (mottling). Other manifestations may also be observed such as a decrease in platelet levels, heart valve abnormalities, and renal or brain diseases. Anticoagulant treatment must be instituted as soon as thrombosis is diagnosed. Given the important risk of recurrence of thrombosis, it is recommended that patients with APS be given long-term preventive antithrombotic treatment, which is often administered for life in the absence of contraindications. Venous thrombosis is treated with vitamin K antagonists (VKA) with a target INR between 2 and 3. Management of ischemic stroke is the subject of discussion, although most teams propose VKA treatment with a target INR > 3.

Synonyms for the disease: Hughes Syndrome, Soulier-Boffa’s Syndrome, Sneddon’s syndrome