Catastrophic antiphospholipid syndrome (CAPS) is a severe condition with high mortality. Since its description in 1992, an important effort has been made to improve and disseminate knowledge on CAPS. Most of our current knowledge comes from the studies performed using the CAPS Registry, a database created in 2000 to gather as many cases as possible in order to better define this disease. It has demonstrated that this condition has multiple faces and is often triggered by a precipitating factor that leads to a thrombotic microangiopathy and cytokine storm involving almost any organ of the body. Analysis of the CAPS Registry has also shown that patients receiving anticoagulation, glucocorticoids and plasma exchange and/or IVIG have a better prognosis. However, there are still many unresolved questions. In this review we summarize what is known and what is still a matter of research in this condition.
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Pediatric Rheumatology Department, Hospital Para El Niño Poblano, Puebla, Mexico.
A female patient in middle childhood was diagnosed with coarctation of the aorta at one month of age and underwent a successful cortectomy. At 11 years old, she developed re-coarctation, which was managed through interventional cardiology. Shortly after the procedure, she experienced a sudden and severe clinical decline, presenting with hypoperfusion of the lower extremities, gastrointestinal bleeding, acute kidney injury, and pancreatitis.
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The Heart Institute, Cincinnati Children's Hospital Medical Center, Cincinnati, OH, USA.
Article Synopsis
- A 16-year-old female, who had been diagnosed with bilateral sub-segmental pulmonary emboli, experienced cardiogenic shock due to weakened heart function.
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A variety of autoimmune disorders are associated with an increased risk of thrombosis. Previous studies have suggested combined therapy of heparin and therapeutic plasma exchange (TPE) with fresh frozen plasma (FFP) as the replacement fluid is beneficial in some cases of acute flare-up of autoimmune diseases complicated by thrombotic events. Nevertheless, it remains unknown whether clinicians do more harm than good by exposing patients to a "thrombotic storm" through simultaneous administration of heparin and the clotting factors in the FFP during TPE.
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Division of Nephrology, Department of Medicine, University of Illinois at Chicago, Chicago, Illinois, USA.
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View Article and Find Full Text PDFCase Report: Primary catastrophic antiphospholipid syndrome in a pediatric patient with cerebral venous sinus thrombosis as the first manifestation.
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Background: Antiphospholipid syndrome (APS) is an autoimmune disease characterized by recurrent vascular thrombotic events. Catastrophic APS (CAPS), which can result in multiple organ failure and even death, is the most severe manifestation of APS. Herein, we report the case of a pediatric patient with CAPS, including the clinical course, diagnosis, and treatment, with the goal of expanding the literature on this condition, as reports of CAPS in pediatric patients are rare.
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