Budd-Chiari syndrome in Behçet's disease: a retrospective multicenter study

Area of Science:

• Vascular Medicine
• Rheumatology
• Hepatology

Background:

• Behçet's disease (BD) is a multisystemic inflammatory disorder.
• Budd-Chiari syndrome (BCS) is a rare complication of BD, characterized by hepatic venous outflow obstruction.

Purpose of the Study:

• To compare clinical features, laboratory findings, and prognosis of BD patients with and without BCS.
• To identify risk factors for mortality in BD patients with BCS.

Main Methods:

• A multicenter retrospective study involving 61 BD patients with BCS and 169 BD patients without BCS.
• Data collected from 1990 to 2017 across 22 centers in Turkey.

Main Results:

• BD patients with BCS had earlier disease onset and diagnosis compared to controls.
• Inferior vena cava (IVC) thrombosis was more frequent in BD-BCS patients.
• Mortality was significantly higher in BD-BCS patients with IVC thrombosis (14.8%) compared to controls.

Conclusions:

• BD-associated BCS presents with earlier onset and higher mortality, particularly with IVC involvement.
• Chronic and silent forms of BD-associated BCS may have a better prognosis.
• Treatment primarily involves immunosuppressants, with anticoagulant therapy remaining controversial.