Article Text
Abstract
Treatment of thrombosis in patients with antiphospholipid syndrome (APS) is based on long-term oral anticoagulation and treatment of obstetric manifestations on the use of aspirin and heparin. These recommendations are based on randomized controlled trials and observational studies. In detail, patients with definite APS with first venous thrombosis have to be treated with prolonged oral anticoagulation at a target international normalized ratio (INR) of 2.0–3.0. Anticoagulation at INR of 3.0–4.0, isolated antiaggregation, anticoagulation at INR 2.0–3.0 or anticoagulation at INR 2.0–3.0 plus antiaggregation have been proposed for definite APS patients with arterial thrombosis. Regarding obstetric APS, although combined therapy with low-dose aspirin and low-molecular-weight heparin is the mainstay of treatment in women with obstetric APS, the strength of evidence of its efficacy is under discussion.
However, there are many grey areas in the field of APS where the evidence is scarce and where the management of certain patients is difficult. Some examples are patients with “seronegative” APS, those who do not display formal (clinical or laboratory) classification criteria for APS, those with refractory APS despite optimal treatment (recurrent thrombotic events despite optimal anticoagulation or recurrent fetal losses despite the combination of aspirin and low molecular weight heparin), and the treatment of clinical manifestations not included in the classification criteria, such as hematologic manifestations (thrombocytopenia and haemolytic anemia), neurologic manifestations (chorea, myelitis or multiple sclerosis-like lesions), nephropathy and heart valve disease associated with antiphospholipid antibodies. In these cases, the recommendations are based on the common sense since the published evidence is scarce, or it does not exist.
In cases of catastrophic APS, an aggressive treatment is required. Therefore, early diagnosis is very important to start adequate therapy and decrease the high mortality rate of these patients. Once the diagnosis is made or suspected, searching and treating the precipitating factor, mainly infection, is the first step of treatment. The specific therapy of catastrophic APS is the combination of anticoagulation with heparin, and corticosteroids as first line of treatment. Additionally adding intravenous immunoglobulins and/or plasma exchange have to be considered in life-threatening cases. In patients with associated SLE, intravenous cyclophosphamide has demonstrated be beneficial. In refractory cases, rituximab or eculizumab should be added.
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