Résumé
Catastrophic antiphospholipid syndrome (CAPLS) was diagnosed in a 64-year-old male who was admitted to our hospital with dyspnea. The clinical and radiological examinations showed pulmonary thromboembolism, and so thromboembolectomy was performed. Abdominal distention rapidly developed several days later, and the abdominal computed tomography (CT) abdominal scan revealed thrombus within the superior mesenteric artery with small bowel and gall bladder distension. Cholecystectomy and jejunoileostomy were performed, and gall bladder necrosis and small bowel infarction were confirmed. The anticardiolipin antibody was positive. Anticoagulant agents and steroids were administered, but the patient expired 4 weeks after surgery due to acute respiratory distress syndrome (ARDS). We report here on a case of catastrophic APLS with manifestations of pulmonary thromboembolism, rapidly progressing GB necrosis and bowel infarction.
Sujets)
Humains , Mâle , Adulte d'âge moyen , Anticorps anticardiolipines , Anticoagulants , Syndrome des anticorps antiphospholipides , Cholécystectomie , Dyspnée , Infarctus , Artère mésentérique supérieure , Nécrose , Embolie pulmonaire , , Stéroïdes , Thrombose , Vessie urinaireRésumé
Catastrophic antiphospholipid syndrome (CAPLS) was diagnosed in a 64-year-old male who was admitted to our hospital with dyspnea. The clinical and radiological examinations showed pulmonary thromboembolism, and so thromboembolectomy was performed. Abdominal distention rapidly developed several days later, and the abdominal computed tomography (CT) abdominal scan revealed thrombus within the superior mesenteric artery with small bowel and gall bladder distension. Cholecystectomy and jejunoileostomy were performed, and gall bladder necrosis and small bowel infarction were confirmed. The anticardiolipin antibody was positive. Anticoagulant agents and steroids were administered, but the patient expired 4 weeks after surgery due to acute respiratory distress syndrome (ARDS). We report here on a case of catastrophic APLS with manifestations of pulmonary thromboembolism, rapidly progressing GB necrosis and bowel infarction.
Sujets)
Humains , Mâle , Adulte d'âge moyen , Anticorps anticardiolipines , Anticoagulants , Syndrome des anticorps antiphospholipides , Cholécystectomie , Dyspnée , Infarctus , Artère mésentérique supérieure , Nécrose , Embolie pulmonaire , , Stéroïdes , Thrombose , Vessie urinaireRésumé
Systemic lupus erythematosus (SLE) is autoimmune disease that often develops antiphopholipid syndrome (APS). Lupus anticoagulant and anticardiolipin antibodies are the hallmarks of APS. The hypercoagulable state in APS may lead to adrenal vein thrombosis, and subsquently lead to hemorrhagic necrosis of the adrenal gland. Adrenal hemorrhage is a very rare complication of APS. Although there have been some reports about adrenal hemorrhage associated with primary APS, adrenal hemorrhage associated secondary APS in SLE has not yet been reported. We describe the adrenal hemorrhage associated with secondary APS in SLE, and the patient which complained of general weakeness, nausea, vomiting and diffuse abdominal pain. Abdominal magnetic resonance imaging (MRI) showed hemorrhage, of both renal glands, and clinical features and immunological studies were consistent with APS in SLE. The acute adrenal insufficiency was much improved after the patient was treated with glucocorticoids