Catastrophic antiphospholipid antibody syndrome. 2001

D S Shames, and P A Broderick
Department of Medicine, Stamford Hospital, Stamford, USA.

The antiphospholipid antibody syndrome may be associated with connective tissue disorders. We describe a patient with catastrophic antiphospholipid antibody syndrome secondary to systemic lupus erythematosus who presented with recurrent abdominal symptoms. Our patient died, and autopsy revealed hemorrhagic infarction of bowel with numerous mesenteric hemorrhages. The treatment for antiphospholipid antibody syndrome ranges from aspirin, warfarin, and plasmapheresis to prednisone and hydroxychloroquine.

UI MeSH Term Description Entries
D008180 Lupus Erythematosus, Systemic A chronic, relapsing, inflammatory, and often febrile multisystemic disorder of connective tissue, characterized principally by involvement of the skin, joints, kidneys, and serosal membranes. It is of unknown etiology, but is thought to represent a failure of the regulatory mechanisms of the autoimmune system. The disease is marked by a wide range of system dysfunctions, an elevated erythrocyte sedimentation rate, and the formation of LE cells in the blood or bone marrow. Libman-Sacks Disease,Lupus Erythematosus Disseminatus,Systemic Lupus Erythematosus,Disease, Libman-Sacks,Libman Sacks Disease
D005260 Female Females
D006801 Humans Members of the species Homo sapiens. Homo sapiens,Man (Taxonomy),Human,Man, Modern,Modern Man
D000328 Adult A person having attained full growth or maturity. Adults are of 19 through 44 years of age. For a person between 19 and 24 years of age, YOUNG ADULT is available. Adults
D016736 Antiphospholipid Syndrome The presence of antibodies directed against phospholipids (ANTIBODIES, ANTIPHOSPHOLIPID). The condition is associated with a variety of diseases, notably systemic lupus erythematosus and other connective tissue diseases, thrombopenia, and arterial or venous thromboses. In pregnancy it can cause abortion. Of the phospholipids, the cardiolipins show markedly elevated levels of anticardiolipin antibodies (ANTIBODIES, ANTICARDIOLIPIN). Present also are high levels of lupus anticoagulant (LUPUS COAGULATION INHIBITOR). Anti-Phospholipid Antibody Syndrome,Anti-Phospholipid Syndrome,Antiphospholipid Antibody Syndrome,Hughes Syndrome,Anti Phospholipid Antibody Syndrome,Anti Phospholipid Syndrome,Antibody Syndrome, Anti-Phospholipid,Antibody Syndrome, Antiphospholipid,Antiphospholipid Antibody Syndromes,Syndrome, Anti-Phospholipid,Syndrome, Anti-Phospholipid Antibody,Syndrome, Antiphospholipid,Syndrome, Antiphospholipid Antibody,Syndrome, Hughes
D017809 Fatal Outcome Death resulting from the presence of a disease in an individual, as shown by a single case report or a limited number of patients. This should be differentiated from DEATH, the physiological cessation of life and from MORTALITY, an epidemiological or statistical concept. Fatal Outcomes,Outcome, Fatal,Outcomes, Fatal

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