Makatsariya A, Asherson R A, Bitsadze V, Baimuradova S, Akinshina S
Department of Obstetrics and Gynecology of Moscow Medical Academy, Noviy Arbat 7, 119017 Moscow, Russia.
Autoimmun Rev. 2006 Dec;6(2):89-93. doi: 10.1016/j.autrev.2006.06.011. Epub 2006 Jul 24.
Catastrophic antiphospholipid syndrome (CAPS) (Asherson's Syndrome), is a life-threatening condition characterized by a rapidly progressive thromboses resulting in a multiorgan dysfunction syndrome (MODS), evidence of systemic inflammatory response syndrome (SIRS) in the presence of antiphospholipid antibodies. CAPS differs from the classic APS by predominantly affecting small vessels, involvement of unusual organs, rapid onset of MODS, and the development of acute respiratory distress syndrome (ARDS) in 25% of patients, which is a feature of SIRS. Obstetric-related multiorgan failure may be a feature of a subset of CAPS more frequently than was previously thought. Patients with obstetric complications should be tested for antiphospholipid antibodies and genetic thrombophilia in order to institute early prophylaxis. Low-molecular-weight heparin is the drug of choice for preventing obstetric complications and CAPS due to its anticoagulant and anti-inflammatory properties.
灾难性抗磷脂综合征(CAPS)(阿舍森综合征)是一种危及生命的疾病,其特征是迅速进展的血栓形成,导致多器官功能障碍综合征(MODS),在存在抗磷脂抗体的情况下出现全身炎症反应综合征(SIRS)的证据。CAPS与经典抗磷脂综合征不同,主要影响小血管,累及不常见器官,MODS起病迅速,25%的患者会出现急性呼吸窘迫综合征(ARDS),这是SIRS的一个特征。产科相关的多器官衰竭可能比以前认为的更常见于CAPS的一个亚组。患有产科并发症的患者应检测抗磷脂抗体和遗传性易栓症,以便尽早进行预防。低分子量肝素因其抗凝和抗炎特性,是预防产科并发症和CAPS的首选药物。