Intravenous immunoglobulin may be of some benefit and cyclophosphamide may be considered in selected cases.
Catastrophic antiphospholipid syndrome (CAPS) is an uncommon kind of antiphospholipid syndrome resulting in multiorgan bankruptcy with a mortality pace of about 50. The syndrome occurs in patients with either systemic lupus erythematosus and new rheumatic diseases (systemic sclerosis, rheumatoid arthritis, main Sjogren syndrome) or only. Antiphospholipid syndrome (APS or APLS) or antiphospholipid antibody syndrome is a disorder of coagulation, which causes blood clots (thrombosis) in both arteries and veins. CAPS causes the blood's clotting mechanism to get haywire, blocking blood flowing to organs throughout the system.
CAPS seems to be a more harmful cousin of a circumstance called antiphospholipid syndrome or APLS. Like CAPS, APLS causes over-coagulation of the blood, that is overly more clotting, which leads to arterial blockages, blood platelet output problems, and, in pregnant women, unscripted miscarriage. CAPS has a mortality pace of about 50. With the formation of a CAPS-Registry more has been learned about this syndrome. Infection, injury, medicine, or operation can be identified in about half the cases as a “trigger. It is thought that cytokines are activated leading to a cytokine tempest with the possibly deadly consequences of organ bankruptcy.
CAPS affects many region of system. The kidney is the almost popular objective. CAPS sufferers frequently come at the hospital with severe kidney symptoms such as reduced or non-existent urine output and higher blood force. CAPS frequently causes substantial worsening in mind role, ranging from disarray to coma. Approximately 25% of reported cases of CAPS affect strained respiration and a nonproductive coughing. People with CAPS may produce intense left ventricular bankruptcy , caused by blockages in the smaller arteries around the eye, as easily as accelerated hypertension and cerebral hemorrhaging.
Treatment most commonly includes anticoagulation and steroids. Plasmapheresis and intravenous immunoglobulin have also been used. Plasmapheris is a procedure that lowers the amount of plasma in the blood by removing whole blood, centrifuging it to separate the plasma from other blood components and then replacing the plasma with a saline solution, fresh frozen plasma or another liquid and then returning the solution to the patient. Women with recurrent miscarriage are often advised to take aspirin.
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