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A case of Budd-Chiari syndrome with Behcet's disease and oral contraceptive usage
Türkay Akbaş1, Neşe Imeryüz, Fatih Bayalan
1Department of Internal Medicine and Critical Care Unit, Marmara University School of Medicine, Istanbul, Turkey.
Insights
Budd-Chiari syndrome (BCS) in a Behcet's disease (BD) patient was linked to unprescribed oral contraceptive (OC) use, leading to shock and death. This highlights the critical need to investigate multiple risk factors in BCS.
Area of Science:
- Hepatology
- Vascular Medicine
- Rheumatology
Background:
- Budd-Chiari syndrome (BCS) is a rare condition characterized by hepatic venous outflow obstruction.
- Behcet's disease (BD) is a multisystemic inflammatory disorder associated with a hypercoagulable state.
- Oral contraceptives (OCs) are known risk factors for venous thromboembolism.
Observation:
- A 33-year-old female with known BD presented with shock, abdominal pain, distention, nausea, vomiting, and confusion.
- Imaging revealed inferior vena cava (IVC) obstruction and extensive hepatic vein thrombosis, consistent with BCS.
- The patient had been using OCs for one year without her physician's knowledge.
Findings:
- The patient was diagnosed with BCS precipitated by a combination of BD and undisclosed OC use.
- Initial treatment included pulse methylprednisolone for BD vasculitis and attempted thrombolytic therapy.
- Despite interventions, the patient's condition deteriorated, leading to hepatic failure and death while awaiting liver transplantation.
Implications:
- This case underscores the multifactorial nature of BCS and the importance of identifying all potential thrombotic risk factors.
- Clinicians should thoroughly question patients with thrombotic conditions like BD about concurrent OC use.
- Early recognition and management of combined risk factors may improve outcomes in BCS patients.
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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