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Behcet's disease in Budd-Chiari syndrome
Anne Claire Desbois1, Pierre Emmanuel Rautou2, Lucie Biard3
1Department of Internal Medicine and clinical Immunology APHP, Paris France, Centre de référence des maladies autoimmunes et systémiques rares, Université Pierre et Marie Curie, Paris 6, Paris, France ; Laboratory I3 "Immunology, Immunopathology, Immunotherapy", UMR CNRS 7211, INSERM U959, Groupe Hospitalier Pitié-Salpetrière, DHU I2B Inflammation, Immunopathology, Biotherapy, Université Pierre et Marie Curie, Paris 6, Paris, France.
Insights
Behcet's disease (BD) is linked to Budd-Chiari syndrome (BCS), often causing inferior vena cava obstruction. Early treatment with anticoagulation and immunosuppressants is recommended for managing BCS in BD patients.
Area of Science:
- Vascular Medicine
- Rheumatology
- Hepatology
Background:
- Behcet's disease (BD) is a known cause of Budd-Chiari syndrome (BCS).
- Limited data exists on the clinical presentation and outcomes of BCS specifically related to BD.
Purpose of the Study:
- To investigate the characteristics and outcomes of patients with both Behcet's disease and Budd-Chiari syndrome.
- To compare these patients with those who have BCS but not BD.
Main Methods:
- Retrospective analysis of 14 patients with co-existing BD and BCS.
- Comparison with a control group of 92 patients with BCS alone.
Main Results:
- Patients with BD and BCS showed higher rates of male gender, North African origin, and inferior vena cava obstruction.
- Higher C-reactive protein levels were observed in the BD-BCS group.
- Medical management including anticoagulation and immunosuppressants was frequently employed and often sufficient, reducing the need for invasive procedures like TIPS.
Conclusions:
- Budd-Chiari syndrome in Behcet's disease patients frequently involves inferior vena cava obstruction.
- Anticoagulation and immunosuppressive therapy are effective in managing BCS symptoms in BD patients.
- Early initiation of immunosuppressive and anticoagulation therapy is the preferred treatment strategy for BCS in the context of BD.
Background:
Behcet's disease (BD) is a well-known cause of Budd-Chiari syndrome (BCS). Data are lacking on the presentation and outcome of BCS related to BD.
Methods:
We investigated the relationship between BD and BCS in 14 patients with both diseases and compared the results to 92 BCS patients without BD.
Results:
Male gender (p = 0.003), North African origin (P = 0.007) and inferior vena cava obstruction (P < 0.0001) were more frequent in patients with BD and BCS than in those with BCS alone and the plasma C-reactive protein level was higher (p = 0.003). Two of the patients with the combined diseases underwent recanalization of the vena cava and the hepatic veins, none received transjugular intrahepatic portosystemic shunts (TIPS), one received a surgical shunt and one underwent liver transplantation. TIPS were less frequent in patients with BD and BCS than in those with BCS alone (P = 0.019). Eighty six per cent of patients with BCS and BD received corticosteroids and immunosuppressive therapy. The 5-year transplantation-free survival rate was 63% in patients with BCS alone and 91% in those without BD (P = 0.11). In our series and in the literature, a high number of patients [12 (61.5%) and 11 (64.7%) respectively] treated with anticoagulation and corticosteroids and/or immunosuppressants did not require invasive treatment.
Conclusion:
This study shows a higher frequency of IVC obstruction in patients with BCS and BD. Medical treatment with anticoagulation and immunosuppressive agents may improve the symptoms of BCS. Therefore early management with immunosuppressive and anticoagulation therapy appears to be the treatment of choice in patients with BCS and BD.
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