Catastrophic antiphospholipid syndrome (CAPS), also known as Ashersons 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.
Specific therapy includes the use of  intravenous heparin and corticosteroids, and possibly plasma exchanges, intravenous immunoglobulin. As a disease associated with high morbidity and mortality, CAPS requires an aggressive multidisciplinary treatment strategy. The following treatments have been used in various combinations: anticoagulation, glucocorticoids, plasma exchange, cyclophosphamide, intravenous immunoglobulins, and anti-platelet agents. Additional steps may have to be taken to manage circulatory problems, kidney failure, and respiratory distress. Recent investigational treatment therapies include high doses of Rituxan (Rituximab) and eculizumab, which are both humanized monoclonal antibodies that target B cell malignancies and prevent C5 complement cleavage, respectively. References


== External links ==