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Orphanet Journal of Rare Diseases
Published in: Orphanet Journal of Rare Diseases 1/2014

Open Access 01-12-2014 | Research article

Behcet’s disease in budd-chiari syndrome

Authors: Anne Claire Desbois, Pierre Emmanuel Rautou, Lucie Biard, Nadia Belmatoug, Bertrand Wechsler, Mathieu Resche-Rigon, Virginie Zarrouk, Bruno Fantin, M Pineton de Chambrun, Patrice Cacoub, Dominique Valla, David Saadoun, Aurélie Plessier

Published in: Orphanet Journal of Rare Diseases | Issue 1/2014

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Abstract

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.
Appendix
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Metadata
Title
Behcet’s disease in budd-chiari syndrome
Authors
Anne Claire Desbois
Pierre Emmanuel Rautou
Lucie Biard
Nadia Belmatoug
Bertrand Wechsler
Mathieu Resche-Rigon
Virginie Zarrouk
Bruno Fantin
M Pineton de Chambrun
Patrice Cacoub
Dominique Valla
David Saadoun
Aurélie Plessier
Publication date
01-12-2014
Publisher
BioMed Central
Published in
Orphanet Journal of Rare Diseases / Issue 1/2014
Electronic ISSN: 1750-1172
DOI
https://doi.org/10.1186/s13023-014-0153-1

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