Abstract

Background: Antiphospholipid syndrome (APS) is an acquired, immune-mediated thrombophilia occurring alone (primary APS, PAPS) or in association with other autoimmune diseases, mainly systemic lupus erythematous (SLE), (secondary APS), characterized by recurrent venous or arterial thrombosis and /or pregnancy morbidity in association with antiphospholipid antibodies (aPL) and/or lupus anticoagulant (LA). Case report: A 36-year-old woman was admitted to the hospital because of acute renal failure and thrombocytopenia. This woman presented with an acute illness characterized by a prodrome of respiratory symptoms and fever that were unresponsive to antibiotic therapy, followed by the progressive involvement of multiple organs. There was enzymatic and functional evidence of myocardial necrosis leading to intermittent, severe heart failure, as well as acute renal failure requiring dialysis, laboratory evidence of pancreatic injury, pulmonary infiltrates with respiratory failure, and central nervous system involvement, with confusion. After a renal biopsy was detected a perinephric hematoma and thrombocytopenia. The laboratory criteria for the diagnosis of the antiphospholipid-antibody syndrome are an elevated value for IgG anticardiolipin antibody, a positive test for lupus anticoagulant, and an elevated value for IgM anticardiolipin antibody. Conclusions: This patient has the antiphospholipid antibody syndrome, with an acute thrombotic angiopathy that caused ischemic damage in the myocardium, pancreas, kidneys and lungs. The renal interstitial inflammation is more severe than that expected from the ischemic injury alone and many reflect either a hypersensitivity drug reaction or in view of the dry Sjogren’s syndrome

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