Department of Obstetrics, Gynecology and Perinatal Medicine, N. F. Filatov Clinical Institute of Children's Health, I. M. Sechenov First Moscow State Medical University (Sechenov University), Trubetskaya Str. 8-2, 119991 Moscow, Russia.
Faculty of Pharmaceutical and Biological Sciences, Montpellier University, 34093 Montpellier, France.
Int J Mol Sci. 2024 Jan 4;25(1):668. doi: 10.3390/ijms25010668.
Unlike classic APS, CAPS causes multiple microthrombosis due to an increased inflammatory response, known as a "thrombotic storm". CAPS typically develops after infection, trauma, or surgery and begins with the following symptoms: fever, thrombocytopenia, muscle weakness, visual and cognitive disturbances, abdominal pain, renal failure, and disseminated intravascular coagulation. Although the presence of antiphospholipid antibodies in the blood is one of the diagnostic criteria, the level of these antibodies can fluctuate significantly, which complicates the diagnostic process and can lead to erroneous interpretation of rapidly developing symptoms. Triple therapy is often used to treat CAPS, which includes the use of anticoagulants, plasmapheresis, and high doses of glucocorticosteroids and, in some cases, additional intravenous immunoglobulins. The use of LMWH is recommended as the drug of choice due to its anti-inflammatory and anticoagulant properties. CAPS is a multifactorial disease that requires not only an interdisciplinary approach but also highly qualified medical care, adequate and timely diagnosis, and appropriate prevention in the context of relapse or occurrence of the disease. Improved new clinical protocols and education of medical personnel regarding CAPS can significantly improve the therapeutic approach and reduce mortality rates.
不同于经典的抗磷脂综合征(APS),CAPS 由于炎症反应增加而导致多发微血栓形成,被称为“血栓风暴”。CAPS 通常在感染、创伤或手术后发生,其起始症状包括:发热、血小板减少、肌肉无力、视觉和认知障碍、腹痛、肾衰竭和弥散性血管内凝血。尽管血液中存在抗磷脂抗体是其中一项诊断标准,但这些抗体的水平可能会大幅波动,这使得诊断过程复杂化,并可能导致对迅速发展的症状的错误解读。CAPS 的治疗通常采用三联疗法,包括使用抗凝剂、血浆置换以及大剂量糖皮质激素,在某些情况下还需要额外的静脉注射免疫球蛋白。由于具有抗炎和抗凝特性,低分子肝素(LMWH)被推荐为首选药物。CAPS 是一种多因素疾病,不仅需要多学科的方法,还需要高度专业的医疗护理、充分和及时的诊断以及在疾病复发或发生时进行适当的预防。改进新的临床方案和对 CAPS 的医疗人员教育,可以显著改善治疗方法并降低死亡率。