Catastrophic antiphospholipid syndrome (CAPS), also known as Asherson's syndrome, is a rare autoimmune disease in which widespread, intravascular clotting causes multi-organ failure. The syndrome is caused by antiphospholipid antibodies that target a group of proteins in the body that are associated with phospholipids. These antibodies activate endothelial cells, platelets, and immune cells, ultimately causing a large inflammatory immune response and widespread clotting. CAPS was first described by Ronald Asherson in 1992. The syndrome exhibits thrombotic microangiopathy, multiple organ thromboses, and in some cases tissue necrosis and is considered an extreme or catastrophic variant of the antiphospholipid syndrome. CAPS has a mortality rate of about 50%. With the establishment of a CAPS-Registry more has been learned about this syndrome, but its cause remains unknown. Infection, trauma, medication, and/or surgery can be identified in about half the cases as a "trigger". It is thought that cytokines are activated leading to a cytokine storm with the potentially fatal consequences of organ failure. A low platelet count is a common finding. Presentation Clinically, the syndrome affects at least three organs and may affect many organ systems. The syndrome usually occurs with small vessel thromboses affecting organ systems such as the gastrointestinal tract and manifestations of the acute respiratory distress syndrome (ARDS), a type of systemic inflammatory response syndrome (SIRS). Peripheral thrombosis may be encountered affecting veins and arteries. Intra-abdominal thrombosis may lead to pain. The body contains β2-GPI, a glycoprotein in the blood, which is considered a natural anticoagulant due to its inhibitory effects on certain aspects of platelet synthesis and function. Because the formation of anti-β2-GPI antibodies can occur after exposure to bacteria, the body favors a hypercoagulable state, which has been noted to activate toll-like receptor 4 resulting in what is known as a cytokine storm or a thrombotic storm. A thrombotic storm may also occur due to the following precipitating events: alterations in coagulation and fibrinolysis, which induce high mortality rates, and infections amongst pediatric patients where IgM and IgG anti-β2-GPI antibodies induce an endothelial signal, leading to a procoagulant state. It is also hypothesized that thrombotic storms occur due to prothrombotic genetic risk factors, which trigger a sped-up form of thrombosis after its first occurrence, rather than being caused solely by environmental factors. Cardiovascular, nervous, kidney, and lung system complications are common. More specifically with the heart, Asherson's syndrome can lead to complications such as mitral valve regurgitation (MVR) in