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A case of antiphospholipid syndrome presenting cryptogenically as Budd-Chiari syndrome, then fulminantly as Libman-Sacks endocarditis
  1. Hart A Goldhar1,
  2. Paloma O’Meara2 and
  3. Lana A Castellucci3
  1. 1 Department of Medicine, University of Ottawa, Ottawa, Ontario, Canada
  2. 2 Department of Medicine, The Ottawa Hospital, Ottawa, Ontario, Canada
  3. 3 Department of Medicine, Division of Hematology, Ottawa Hospital Research Institute, University of Ottawa, The Ottawa Hospital, Ottawa, Ontario, Canada
  1. Correspondence to Dr Hart A Goldhar, hgoldhar{at}


A 58 year-old left-handed woman was transferred to our hospital with an evolving left middle cerebral artery stroke, severe thrombocytopenia and elevated inflammatory markers. She had a history of chronic Budd-Chiari syndrome (BCS) 16 months prior, attributed to a calcified web in the inferior vena cava that was stented. No thrombophilia testing was performed at that time. The current presentation demonstrated dense right-sided facial and arm paresis and neglect. Erythrocyte sedimentation rate and C-reactive protein were elevated, an autoimmune workup was consistent with a new diagnosis of systemic lupus erythematosus and triple-positive antiphospholipid antibodies. A transesophageal echocardiogram demonstrated a vegetation consistent with Libman-Sacks endocarditis (LSE), thought to have embolised to the brain. The patient was treated acutely with steroids, intravenous immunoglobulin and clopidogrel. This case demonstrates an atypical constellation of the antiphospholipid syndrome, with a novel presentation of BCS and LSE, and reinforces the importance of hypercoagulability screening in this population.

  • systemic lupus erythematosus
  • venous thromboembolism
  • stroke
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  • Contributors HAG wrote the complete article. LAC and PO refined and proofread the manuscript.

  • Funding The authors have not declared a specific grant for this research from any funding agency in the public, commercial or not-for-profit sectors.

  • Competing interests None declared.

  • Provenance and peer review Not commissioned; externally peer reviewed.

  • Patient consent for publication Next of kin consent obtained.

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