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.

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