Linnemann, Birgit (2018) Antiphospholipid syndrome - an update. VASA-EUROPEAN JOURNAL OF VASCULAR MEDICINE, 47 (6). pp. 451-464. ISSN 0301-1526
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Antiphospholipid syndrome (APS) is an autoantibody-mediated acquired thrombophilia. It is characterized by the presence of antiphospholipid antibodies (APL) that are directed against phospholipid-binding plasma proteins, such as beta-2-glycoprotein I (b2GPI). Its main manifestations are recurrent vascular thromboses (so-called "thrombotic APS") and pregnancy complications ("obstetric APS"). According to the current consensus criteria, a persistently positive functional lupus anticoagulant (LA) assay and/or the presence of anti-b2GPI and/or anti-cardiolipin antibodies, together with clinical symptoms, is mandatory for the diagnosis of APS. Other clinical features, such as thrombocytopenia, Coombs-positive haemolytic anaemia, heart valve disease, renal microangiopathy and neurologic disorders are also common in APL-positive patients. APS can be associated with other autoimmune disorders, such as systemic lupus erythematosus. In rare cases, catastrophic APS (CAPS) occurs, with the development of excessive thrombosis at multiple sites, usually affecting small vessels and leading to multi-organ dysfunction and organ failure. Treatment usually comprises antithrombotic therapy using antiplatelet and anticoagulant agents. However, there is no consensus concerning the intensity or duration of therapy. Despite apparently adequate anticoagulation, the risk of recurrent thrombosis remains high. For patients with CAPS, a combined therapeutic approach that includes anticoagulation, glucocorticoids, plasma exchange and/or intravenous immunoglobulin seems to be the best treatment option.
| Item Type: | Article |
|---|---|
| Uncontrolled Keywords: | INTERNATIONAL CONSENSUS STATEMENT; SYSTEMIC-LUPUS-ERYTHEMATOSUS; RECURRENT PREGNANCY LOSS; VITAMIN-K ANTAGONISTS; 1ST THROMBOTIC EVENT; ED AMERICAN-COLLEGE; HIGH-RISK PATIENTS; TASK-FORCE REPORT; CLASSIFICATION CRITERIA; ANTITHROMBOTIC THERAPY; Antiphospholipid syndrome; lupus anticoagulants; anti-cardiolipin; anti-beta-2-glycoprotein I; vascular thrombosis; pregnancy complication |
| Subjects: | 600 Technology > 610 Medical sciences Medicine |
| Divisions: | Medicine > Abteilung für Gefäßchirurgie |
| Depositing User: | Petra Gürster |
| Date Deposited: | 10 Jul 2020 11:50 |
| Last Modified: | 10 Jul 2020 11:50 |
| URI: | https://pred.uni-regensburg.de/id/eprint/13726 |
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