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. 2014 Sep 13:9:104.
doi: 10.1186/s13023-014-0153-1. eCollection 2014.

Behcet's disease in Budd-Chiari syndrome

Anne Claire Desbois  1 , Pierre Emmanuel Rautou  2 , Lucie Biard  3 , Nadia Belmatoug  4 , Bertrand Wechsler  5 , Mathieu Resche-Rigon  3 , Virginie Zarrouk  4 , Bruno Fantin  4 , M Pineton de Chambrun  5 , Patrice Cacoub  6 , Dominique Valla  2 , David Saadoun  6 , Aurélie Plessier  2
Affiliations

Behcet's disease in Budd-Chiari syndrome

Anne Claire Desbois et al. Orphanet J Rare Dis. .

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.

Keywords: Behcet’s disease; Budd-chiari syndrome; Hepatic vein thrombosis; Immunousuppressive agents.

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Figures

Figure 1
Figure 1
Outcome of the 14 BD patients according to treatments (specific treatments of BD and treatments of BCS).
Figure 2
Figure 2
Overall survival in BCS patients with (n = 14) and without (n = 92) BD (a); Transplantation free survival in BCS patients with (n = 14) and without (n = 92) BD (b).
See this image and copyright information in PMC

References

    1. Valla D-C. Primary budd-chiari syndrome. J Hepatol. 2009;50(1):195–203. doi: 10.1016/j.jhep.2008.10.007. - DOI - PubMed
    1. Uskudar O, Akdogan M, Sasmaz N, Yilmaz S, Tola M, Sahin B. Etiology and portal vein thrombosis in budd-chiari syndrome. World J Gastroenterol. 2008;14(18):2858–2862. doi: 10.3748/wjg.14.2858. - DOI - PMC - PubMed
    1. Sakr M, Barakat E, Abdelhakam S, Dabbous H, Yousuf S, Shaker M, Eldorry A. Epidemiological aspects of budd-chiari in Egyptian patients: a single-center study. World J Gastroenterol. 2011;17(42):4704–4710. doi: 10.3748/wjg.v17.i42.4704. - DOI - PMC - PubMed
    1. International Team for the Revision of the International Criteria for Behçet’s Disease (ITR-ICBD) The international criteria for Behçet’s disease (ICBD): a collaborative study of 27 countries on the sensitivity and specificity of the new criteria. J Eur Acad Dermatol Venereol. 2014;28(3):338–347. doi: 10.1111/jdv.12107. - DOI - PubMed
    1. Calamia KT, Schirmer M, Melikoglu M. Major vessel involvement in behçet disease. Curr Opin Rheumatol. 2005;17(1):1–8. doi: 10.1097/01.bor.0000145520.76348.dd. - DOI - PubMed