Abstract
We present a case of Budd–Chiari syndrome (BCS) having two risk factors, Behcet’s disease (BD) and oral contraceptive (OC) usage. A 33-year-old woman with BD was admitted to the Emergency Unit with nausea, vomiting, abdominal pain, abdominal distention, and confusion started 12 days ago before admission. Since the patient was in a shock state, she was taken to the Intensive Care Unit (ICU) with the suspicion of abdomen-originated sepsis. Abdominal ultrasound showed massive hepatosplenomegaly and moderate ascites. Abdominal MRI revealed an inferior vena cava (IVC) obstruction starting above the renal veins and diffuse thrombosis of the right and medial hepatic veins. An extensive thrombosis of the IVC and the hepatic veins (BCS) which led to shock was diagnosed. In addition to BD, the unnotified OC usage for a year by the patient without her doctor’s knowledge was recognized as possible precipitating factor of BCS. Pulse methylprenisolone was started for three consecutive days to treat active BD-induced vasculitis. IVC digital subtraction angiography (DSA) showed occlusion of the IVC below the hepatic veins with extensive collateral circulation originating at the occlusion level suggesting that obliteration had a subacute or chronic course. Since intralesional thrombolytic therapy failed, the patient was transferred to a liver transplantation center. While waiting for an appropriate donor, the patient died due to hepatic failure. Since BCS is mortal and deemed multi-factorial, every patient with a thrombotic risk factor such as BD should be questioned for other possible causes of thrombosis.
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Acknowledgments
T. Akbaş and F. Bayalan cared for the patient during her hospital stay, gathered the raw data of the case and contributed to writing of the paper. N. İmeryüz consulted the patient during hospital stay, contributed to the writing. P. Atagündüz followed the patient at rheumatology outpatients’ clinic and contributed to writing of the paper. F. Baltacıoğlu did the radiological work-up including angiography, prepared figures and contributed to the writing of the paper. H. Direskeneli and L. Mülazımoğlu consulted the patient, reviewed the paper and contributed to the writing of the paper. All authors read, edited and ultimately approved the final manuscript.
All authors have disclosed that they have no financial involvement with any organization or entity. They also have disclosed that this case report has not been published and is not under consideration for publication elsewhere. Financial support has not been taken from any organization or person during the preparation of the writing.
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Akbaş, T., İmeryüz, N., Bayalan, F. et al. A case of Budd–Chiari syndrome with Behcet’s disease and oral contraceptive usage. Rheumatol Int 28, 83–86 (2007). https://doi.org/10.1007/s00296-007-0377-7
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DOI: https://doi.org/10.1007/s00296-007-0377-7