title: Diffuse large and small bowel necrosis in catastrophic antiphospholipid syndrome.
abstract: The catastrophic variant is an accelerated form of the antiphospholipid syndrome resulting in multiorgan failure because of multiple small vessel occlusions. We report a case of catastrophic antiphospholipid syndrome in a patient with subacute cutaneous lupus erythematosus and ischemic bowel, who presented with acute abdominal pain due to diffuse right colon and small bowel necrosis requiring large resection, associated with acute respiratory distress syndrome, thrombocytopenia and disseminated intravascular coagulation. Histopathological examination of resected tissues showed diffuse arteriolar and venous thrombosis but no vasculitis, and mesenteric artery lumen severely narrowed by intimal fibrosis. The patient died 15 days after admission despite treatment with anticoagulation, steroids, continuous hemofiltration and plasma exchange. Ischemic bowel and diffuse intestinal necrosis may be secondary to the antiphospholipid syndrome, and a high level of suspicion and an early diagnosis are required.
authors.affiliation: Department of Internal Medicine, CHU Tenon (AP-HP), Paris, France. claude.bachmeyer@tnn.aphp.fr
authors.forename: Claude
authors.initials: C
authors.lastname: Bachmeyer
authors.forename: Alain
authors.initials: A
authors.lastname: Barrier
authors.forename: Aline
authors.initials: A
authors.lastname: Frazier
authors.forename: Jean-Pierre
authors.initials: JP
authors.lastname: Fulgencio
authors.forename: Isabelle
authors.initials: I
authors.lastname: Lecomte
authors.forename: Gilles
authors.initials: G
authors.lastname: Grateau
authors.forename: Patrice
authors.initials: P
authors.lastname: Callard
mesh_terms: D000368:Aged; D016736:Antiphospholipid Syndrome / Q000150:complications*; D002388:Catastrophic Illness; D003106:Colon / Q000473:pathology*; D017809:Fatal Outcome; D005260:Female; D006801:Humans; D007421:Intestine, Small / Q000473:pathology*; D008180:Lupus Erythematosus, Systemic / Q000150:complications*; D009336:Necrosis / Q000209:etiology