Budd-Chiari syndrome associated with Behçet's disease

Area of Science:

• Vascular Medicine
• Hepatology
• Rheumatology

Background:

• Budd-Chiari syndrome (BCS) is a rare but severe hepatic vein occlusion complication in Behçet's disease (BD).
• Understanding BCS prevalence and clinical characteristics in BD is crucial for patient management.

Purpose of the Study:

• To determine the prevalence of BCS in BD patients.
• To analyze the clinical and laboratory findings, treatment strategies, and clinical course of BCS associated with BD.

Main Methods:

• Retrospective analysis of 220 patients diagnosed with BD.
• Selection and detailed analysis of patients diagnosed with BCS, including epidemiological, clinical imaging, and outcome data.

Main Results:

• BCS was identified in 7 male BD patients (3.2% prevalence), with a subacute to chronic clinical course.
• Hepatic vein thrombosis frequently co-occurred with inferior vena cava (IVC) thrombosis (6/7 cases) and other venous thromboses (4/7 cases).
• Anticoagulation and high-dose corticotherapy, sometimes with cyclophosphamide, were primary treatments; 6/7 patients had favorable outcomes, with one mortality due to hepatic failure.

Conclusions:

• BCS is a significant complication in BD patients, with a prevalence of 3.2%.
• IVC involvement and associated venous thromboses are common in BCS-BD.
• Prognosis is favorable with timely medical interventions, including anticoagulation and vasculitis treatment.