Khizroeva J, Bitsadze V, Makatsariya A
a Department of Obstetrics and Gynecology #2 , Faculty of Preventive Medicine of I.M. Sechenov First Moscow State Medical University (Sechenov University) , Moscow , Russia.
J Matern Fetal Neonatal Med. 2019 Jun;32(12):2091-2094. doi: 10.1080/14767058.2017.1422715. Epub 2018 Jan 8.
We have observed the development of a catastrophic antiphospholipid syndrome (CAPS) in a pregnant woman hospitalized at 28 weeks of gestation with a severe preeclampsia. On the same day, an eclampsia attack developed, and an emergency surgical delivery was performed. On the third day, multiorgan failure developed. Examination showed a persistent circulation of lupus anticoagulant, high level of antibodies to cardiolipin, b2-glycoprotein I, and prothrombin. The usual diagnosis of the severe preeclampsia masked a catastrophic antiphospholipid syndrome, exacerbated by the coincident presence of several types of antiphospholipid antibodies. The first pregnancy resulted in a premature birth at 25 weeks, possibly also due to the circulation of antiphospholipid antibodies. The trigger of the catastrophic form development was the pregnancy itself, surgical intervention, and hyperhomocysteinemia. CAPS is the most severe form of antiphospholipid syndrome, manifested in multiple microthrombosis of microcirculation of vital organs and in the development of multiorgan failure against the background of the high level of antiphospholipid antibodies. CAPS is characterized by renal, cerebral, gastrointestinal, adrenal, ovarian, skin, and other forms of microthrombosis. Thrombosis recurrence is typical. Thrombotic microvasculopathy lies at the heart of multiorgan failure and manifests clinically in central nervous system lesions, adrenal insufficiency, and ARDS development. CAPS is a life-threatening condition, therefore, requires an urgent treatment. Optimal treatment of CAPS is not developed. CAPS represent a general medical multidisciplinary problem.
我们观察到一名妊娠28周因重度子痫前期住院的孕妇发生了灾难性抗磷脂综合征（CAPS）。同一天，子痫发作，遂行急诊手术分娩。第三天，出现多器官功能衰竭。检查显示狼疮抗凝物持续存在、抗心磷脂抗体、β2糖蛋白I抗体及凝血酶原水平升高。重度子痫前期的常见诊断掩盖了灾难性抗磷脂综合征，多种抗磷脂抗体的同时存在使其病情加重。首次妊娠于25周早产，可能也是由于抗磷脂抗体的存在。灾难性抗磷脂综合征发生的诱因是妊娠本身、手术干预及高同型半胱氨酸血症。CAPS是抗磷脂综合征最严重的形式，表现为重要器官微循环的多发性微血栓形成及在抗磷脂抗体水平升高背景下多器官功能衰竭的发生。CAPS的特征是肾脏、大脑、胃肠道、肾上腺、卵巢、皮肤及其他形式的微血栓形成。血栓复发很常见。血栓性微血管病是多器官功能衰竭的核心，临床上表现为中枢神经系统病变、肾上腺功能不全及急性呼吸窘迫综合征的发生。CAPS是一种危及生命的疾病，因此需要紧急治疗。目前尚未制定出CAPS的最佳治疗方案。CAPS是一个综合的医学多学科问题。
