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Budd-Chiari syndrome in Behçet's disease: a retrospective multicenter study
Lütfi Akyol1, Bahtiyar Toz2, Özün Bayındır3
1Department of Internal Medicine, Division of Rheumatology, Health Sciences University, Gazi Yaşargil Training and Research Hospital, Diyarbakır, Turkey. drakyol19@gmail.com.
Insights
Behçet's disease (BD) patients with Budd-Chiari syndrome (BCS) experience earlier symptom onset and diagnosis, with higher rates of inferior vena cava (IVC) thrombosis and mortality. Mortality is significantly increased in BD-BCS patients with IVC thrombosis.
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.
Objective:
To compare the clinical features, laboratory findings, and prognosis of Behçet's disease (BD) patients with and without Budd-Chiari syndrome (BCS).
Methods:
This multicenter retrospective study investigated 61 (M/F: 41/20) patients with BD, having coexistent BCS, and 169 (M/F:100/69) BD patients as the control group without BCS from 22 different centers of Turkey diagnosed between 1990 and 2017.
Results:
Of the total 61 BD patients with BCS, the onset of the first symptom and the median age of diagnosis were earlier in contrast to BD patients without BCS (p = 0.005 and p = 0.007). Lower extremity deep vein and inferior vena cava (IVC) thrombosis were more common in patients with BCS (all; p < 0.01) compared to the control group. Mortality was significantly higher in BD-BCS patients with IVC thrombosis than in the controls (p = 0.004). Since most of the cases in our cohort had chronic and silent form of BCS, mortality rate was 14.8%, which was on the lower range of mortality rate reported in literature (14-47%). While all BD-BCS patients received immunosuppressive (IS) agents, only half of them received additional anticoagulant treatments. Among IS agents, interferon treatment was more frequently used in this cohort (19%), compared to other series reported in literature (2.3%).
Conclusion:
To our knowledge, this is the largest series of BD patients with BCS. Our patients had earlier disease onset and diagnosis, higher frequency of IVC thrombosis, and higher mortality rate, compared to BD patients without BCS. Mortality was significantly higher in BD-BCS patients with IVC thrombosis compared to controls. Key Points • Mortality rate is higher in BD-associated BCS patients with IVC involvement. • Chronic and silent form of BD-associated BCS has a better prognosis. • The main treatment options are corticosteroids and immunosuppressive agents, whereas anticoagulant treatment remains controversial.
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