The catastrophic antiphospholipid syndrome, 1998. A review of the clinical features, possible pathogenesis and treatment
The catastrophic antiphospholipid syndrome, 1998. A review of the clinical features, possible pathogenesis and treatment
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DOI：
10.1177/096120339800700214
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发表时间：
1998-01-01
期刊：
影响因子：
2.6
通讯作者：
Asherson, RA
中科院分区：
文献类型：
--
作者：
Asherson, RA
本文回顾了50例表现出灾难性抗磷脂综合征（CAPS）特征的患者。临床特征主要包括器官受累，而不是“单纯性”抗磷脂综合征（APS）患者所见的大血管静脉或动脉闭塞，这使得这种罕见并发症的发病机制可能与APS患者有所不同。这种疾病的死亡率为50%，大多数患者死于心脏和呼吸衰竭。15名患者（28%）还患有弥散性血管内凝血（DIC），这可能是CAPS多器官血栓性微血管病变的特征之一。尽管大多数患者接受了大剂量静脉注射类固醇、肝素、环磷酰胺和其他治疗方式（如静脉注射球蛋白），血浆置换（提倡治疗TTP，一种类似的微血管疾病）似乎提供了一些好处（恢复68%）。全身性炎症反应综合征（SIRS）是15例成人呼吸窘迫综合征（ARDS）的主要临床表现。CAPS的发病机制似乎依赖于已经患有高凝状态的患者的“两次发作”甚至“三次发作”假说。诱发因素包括感染、创伤（手术）、药物管理和华法林停药。最近的一种观点认为，多发血栓病变本身可能有助于进一步血栓形成（“血栓风暴”）也进行了讨论。
A review of 50 patients who manifest features of the catastrophic antiphospholipid syndrome (CAPS) is presented. The clinical features comprise mainly organ involvement as opposed to large-vessel venous or arterial occlusions as is seen in patients with 'simple' antiphospholipid syndrome (APS), which makes the pathogenesis of this unusually rare complication perhaps somewhat different from that of patients with the APS. The mortality of the condition is 50%, most patients dying as a result of a combination of cardiac and respiratory failure. Fifteen patients (28%) suffered from disseminated intravascular coagulation (DIC) as well, which may have contributed to the multiorgan thrombotic microangiopathy characteristic of the CAPS. Although most patients were treated with high-dose i.v. steroids, heparin, cyclophosphamide and other modalities of therapy (such as i.v. globulin), plasmapheresis (advocated for TTP, a similar microangiopathic condition) seemed to offer some benefit (68% recovery). The systemic inflammatory response syndrome (SIRS) was responsible for some of the clinical manifestations such as adult respiratory distress syndrome (ARDS) seen in 15 patients. Pathogenesis of the CAPS seems dependent on a 'two-hit' or even 'three-hit' hypothesis in patients already suffering from a hypercoagulable state. Precipitating factors include infections, trauma (surgical), drug administration and warfarin withdrawal. A recent view that the multiple thrombotic lesions themselves may contribute to further thrombosis ('thrombotic storm') is also discussed.