Catastrophic Antiphospholipid Syndrome
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Published:2024-01-04
Issue:1
Volume:25
Page:668
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ISSN:1422-0067
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Container-title:International Journal of Molecular Sciences
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language:en
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Short-container-title:IJMS
Author:
Bitsadze Victoria1, Yakubova Fidan1, Khizroeva Jamilya1ORCID, Lazarchuk Arina1ORCID, Salnikova Polina1ORCID, Vorobev Alexander1, Tretyakova Maria1, Degtyareva Natalia1ORCID, Grigoreva Kristina1, Gashimova Nilufar1ORCID, Kvaratskheliia Margaret1, Makatsariya Nataliya1, Kudryavtseva Ekaterina1, Tomlenova Anna1, Gris Jean-Christophe12ORCID, Elalamy Ismail134, Ay Cihan15ORCID, Makatsariya Alexander1
Affiliation:
1. 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 2. Faculty of Pharmaceutical and Biological Sciences, Montpellier University, 34093 Montpellier, France 3. Department Hematology and Thrombosis Center, Medicine Sorbonne University, 75012 Paris, France 4. Hospital Tenon, 4 Rue de la Chine, 75020 Paris, France 5. Department of Medicine I, Clinical Division of Hematology and Hemostaseology, Medical University of Vienna, 1080 Vienna, Austria
Abstract
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.
Subject
Inorganic Chemistry,Organic Chemistry,Physical and Theoretical Chemistry,Computer Science Applications,Spectroscopy,Molecular Biology,General Medicine,Catalysis
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