Catastrophic Antiphospholipid Syndrome

Catastrophic Antiphospholipid Syndrome

4 January 2024 | Victoria Bitsadze, Fidan Yakubova, Jamilya Khizirova, Arina Lazarchuk, Polina Salnikova, Alexander Vorobey, Maria Tretyakova, Natalia Degtyareva, Kristina Grigoreva, Nilufar Gashimova, Margaret Kvaratskhelia, Nataliya Makatsariya, Ekaterina Kudryavtseva, Anna Tomlenova, Jean-Christophe Gris, Ismail Elalamy, Cihan Ay and Alexander Makatsariya
Catastrophic Antiphospholipid Syndrome (CAPS) is a rare and life-threatening condition characterized by multiple organ failure and thrombosis, often triggered by infection, trauma, or surgery. It is a severe form of antiphospholipid syndrome (APS), which is an autoimmune disorder involving antiphospholipid antibodies (APLA). CAPS is diagnosed based on clinical and laboratory criteria, including damage to three or more organs, rapid clinical progression, and histopathological evidence of small vessel occlusion. The disease is associated with high mortality, requiring early diagnosis and aggressive treatment. Risk factors include infection, surgery, pregnancy, and genetic thrombophilia. Pathogenesis involves APLA-mediated activation of endothelial cells, immune cells, and platelets, leading to inflammation, coagulation activation, and microthrombosis. Treatment includes anticoagulation, plasmapheresis, corticosteroids, and intravenous immunoglobulins. Prognosis is poor without timely intervention, but improved diagnosis and treatment have reduced mortality. CAPS can also occur in pregnancy, complicating obstetric management. The condition is associated with systemic inflammation and thrombosis, and its management requires an interdisciplinary approach. Recent studies highlight the role of SARS-CoV-2 in triggering CAPS through hyperinflammatory responses and complement activation. Prevention strategies include anticoagulation, monitoring for risk factors, and early intervention in high-risk patients. Effective management of CAPS requires a combination of therapies and close monitoring to improve outcomes.Catastrophic Antiphospholipid Syndrome (CAPS) is a rare and life-threatening condition characterized by multiple organ failure and thrombosis, often triggered by infection, trauma, or surgery. It is a severe form of antiphospholipid syndrome (APS), which is an autoimmune disorder involving antiphospholipid antibodies (APLA). CAPS is diagnosed based on clinical and laboratory criteria, including damage to three or more organs, rapid clinical progression, and histopathological evidence of small vessel occlusion. The disease is associated with high mortality, requiring early diagnosis and aggressive treatment. Risk factors include infection, surgery, pregnancy, and genetic thrombophilia. Pathogenesis involves APLA-mediated activation of endothelial cells, immune cells, and platelets, leading to inflammation, coagulation activation, and microthrombosis. Treatment includes anticoagulation, plasmapheresis, corticosteroids, and intravenous immunoglobulins. Prognosis is poor without timely intervention, but improved diagnosis and treatment have reduced mortality. CAPS can also occur in pregnancy, complicating obstetric management. The condition is associated with systemic inflammation and thrombosis, and its management requires an interdisciplinary approach. Recent studies highlight the role of SARS-CoV-2 in triggering CAPS through hyperinflammatory responses and complement activation. Prevention strategies include anticoagulation, monitoring for risk factors, and early intervention in high-risk patients. Effective management of CAPS requires a combination of therapies and close monitoring to improve outcomes.
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[slides and audio] Catastrophic Antiphospholipid Syndrome