Diffuse large and small bowel necrosis in catastrophic antiphospholipid syndrome.
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.
Bachmeyer
Claude
C
Department of Internal Medicine, CHU Tenon (AP-HP), Paris, France. claude.bachmeyer@tnn.aphp.fr
Barrier
Alain
A
Frazier
Aline
A
Fulgencio
Jean-Pierre
JP
Lecomte
Isabelle
I
Grateau
Gilles
G
Callard
Patrice
P
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