Disease Info

Disease:  Antiphospholipid Syndrome

Antiphospholipid Syndrome (APS)

Antiphospholipid syndrome (APS) is a systemic autoimmune disease with the highest prevalence in women of childbearing age, characterized by venous and/or arterial thrombosis, pregnancy complications and the presence of specific autoantibodies called antiphospholipid antibodies (aPL). aPL are able to activate the coagulation system; indeed, APS is called sticky blood syndrome because of the increased tendency to form blood clots both in veins and arteries of any organ and system. APS can be primary or can complicate SLE or other autoimmune diseases. One of the most serious complications of APS occurs when clots form in the brain, causing strokes. In a small minority of patients, multi-organ failure, due to diffuse clotting in small vessels and a massive immune response, develops within days, leading to the so-called catastrophic antiphospholipid syndrome (CAPS).

Other symptoms and complications of APS include a characteristic lacy, net-like red skin rash (livaedo reticularis), heart valve abnormalities (Libman Sacks endocarditis), kidney dysfunction and thrombocytopenia. Neurological symptoms include headache, dementia and seizures. Infrequently, individuals may develop chorea (a movement disorder in which the body and limbs writhe uncontrollably), cognitive dysfunction (such as poor memory), transverse myelitis, depression or psychosis, optic neuropathy, or sudden hearing loss. Pregnancy complications include recurrent miscarriages, stillbirth, pre-eclampsia. APS is diagnosed by the presence of aPL positivity together with a history of either arterial or venous thrombosis, or one or more of the aforementioned pregnancy troubles. The therapy is based on aspirin or anticoagulants and hydroxychloroquine; sometimes immunosuppressants are needed.



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