The antiphospholipid syndrome Featured

The antiphospholipid syndrome (APS) is characterized by venous and arterial thromboses as well as obstetric morbidity, in the presence of antiphospholipid antibodies (aPL), namely lupus anticoagulant, anticardiolipin antibodies (aCL) and/or antibodies directed against various proteins, mainly 2 glycoprotein I (2GPI) (Box 1). Among the spectrum of thrombotic disorders associated with APS, deep vein thrombosis is the most frequently reported venous thrombotic manifestation, whereas cerebrovascular accidents are the most common arterial thrombotic manifestations.

In untreated patients with APS, thrombosis recurs in 19-29% of patients per year in the cohort studies reported to date. A systematic review published in 2007 recommended that patients with APS patients should be treated with oral anticoagulants to a target international normalized ratio (INR) of 2.0–3.0 in those with a first-diagnosis of venous thrombosis and to an INR of over 3.0 in those with a first diagnosis of arterial thrombosis.

In 2009, the 5-year-follow-up results of the Euro-Phospholipid Project, a prospective European epidemiologic study designed to identify the main causes of morbidity and mortality in 1000 patients with APS, were released. Over the course of the study, 5.3% of the cohort died, mostly as a result of bacterial infections.

The catastrophic form of APS is characterized by multiple vascular occlusive events mainly affecting small vessels that evolve over a very short period of time (usually, less than 1 week). Patients with catastrophic APS represent less than 1% of diagnosed cases, but they have a high mortality rate of around 50% during the acute event according to the earliest published series. Cerebral involvement is the main cause of death in these patients, being present in 27.2%, followed by cardiac involvement (19.8%) and infection (19.8%). Combined treatment with anticoagulants, corticosteroids and plasma exchanges and/or intravenous immunoglobulins has been associated with a significant decline in the mortality rate.

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Last modified on Sunday, 04 May 2014 21:10

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