Woman with Anti-phospholipid Syndrome

Friday, November 19, 2010
A 52 year old woman, affected by the anti-phospholipid syndrome (APS), and subjected to dialysis because of right shrunken kidney and left nephrectomy, came to our attention for complaints of generalized abdominal pain and frequent episodes of diarrhoea.
Her medical history included coagulation disorders, thromboembolic events, and positioning of aorto-superior mesenteric, left aorto-renal and aorto-bi-iliac prostheses.
Two months earlier, the patient developed an obstruction of aorto-superior mesenteric bypass with distal revascularization by collateral vessels stemming from gastroduodenal artery and severe subocclusive stenosis of left aorto-renal bypass. After left renal revascularization, nephrectomy was carried out owing to acute intraparenchymal bleeding.
Following the onset of abdominal symptoms erect Xray of the abdomen and CT scan were done.



One week later Abdominal CT scan and thoracic CT were done.



What is the most likely diagnosis in this patient?

ANSWER

Plain abdominal X-ray showed gastric distension, with evident gas-fluid levels in the small bowel area, while the colonic frame was empty.CT scan confirmed the above findings, showing diffuse fluid distension of small bowel loops, with multiple gas-fluid levels.
CT scans taken after One week later showed: an inhomogeneous hypodense area located just below the diaphragm and characterized by indistinct margins and dilatation of intrahepatic bile-ducts, suggestive of hepatic abscess. Moreover, a thoracic CT scan revealed a consolidation area within the lower lobe of right lung in association with scissural thickening.

Ischemic small bowel infarction in APS.

APS is an autoimmune disorder, characterized by association of arterial/venous thrombosis or recurrent pregnancy loss with positivity in at least one of the laboratory tests employed to detect anti-phospholipid (aPL) antibodies, including lupus anticoagulant (LA), anti-cardiolipin (aCL) and anti-B2-glycoprotein I (aB-2GPI) antibodies.
Clinical spectrum of APS range from an increased risk of thromboembolic events, including peripheral thrombophlebitis, pulmonary thromboembolism, stroke, retinal artery occlusion, Devic’s syndrome, myocardial infarction, placental thrombosis, Budd syndrome, skin and gastrointestinal disturbances.

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7 comments

  1. Anonymous Says:
  2. SLE

     
  3. Anonymous Says:
  4. Mesentric infarct secondary to APLA

     
  5. Anonymous Says:
  6. multiple infarcts and mesenteric infarct --> ileus --> flatulence

     
  7. Anonymous Says:
  8. aps usually when associated wiz autoimmune diseases,indicate poor prognosis.aps can affect any body organ n system.sometimes diff to treat in severe cases.main criteria- arterial n venous thrombosis,pregnancy morbidity,LAC n ACL antibody positivity.thrombocytopenia also present.lifelong anticoagulation is effective in prevention of 2' thrombosis but care shoulb b taken to balance the intensity of coagulation wiz bleeding risk!

     
  9. Anonymous Says:
  10. Lupus erythematodes systemicus

     
  11. Farshad Says:
  12. Scleroderma.Systemic sclerosis.

     
  13. Anonymous Says:
  14. mesentric infarct

     

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