title: Behcet's disease in Budd-Chiari syndrome.
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.
  
  
  CONCLUSIONS
  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.
authors.affiliation: Department 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.
authors.forename: Anne Claire
authors.initials: AC
authors.lastname: Desbois
authors.affiliation: Department of Hepatology; Hôpital Beaujon, INSERM U773, Service d'hépatologie, 100 boulevard du Général Leclerc, 92118 Clichy cedex, France.
authors.forename: Pierre Emmanuel
authors.initials: PE
authors.lastname: Rautou
authors.affiliation: Department of Biostatistics and Medical Data Processing; INSERM U717, Hôpital Saint-Louis, Paris, France.
authors.forename: Lucie
authors.initials: L
authors.lastname: Biard
authors.affiliation: Department of Internal Medicine, Hôpital Beaujon, Clichy, France.
authors.forename: Nadia
authors.initials: N
authors.lastname: Belmatoug
authors.affiliation: Department 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.
authors.forename: Bertrand
authors.initials: B
authors.lastname: Wechsler
authors.affiliation: Department of Biostatistics and Medical Data Processing; INSERM U717, Hôpital Saint-Louis, Paris, France.
authors.forename: Mathieu
authors.initials: M
authors.lastname: Resche-Rigon
authors.affiliation: Department of Internal Medicine, Hôpital Beaujon, Clichy, France.
authors.forename: Virginie
authors.initials: V
authors.lastname: Zarrouk
authors.affiliation: Department of Internal Medicine, Hôpital Beaujon, Clichy, France.
authors.forename: Bruno
authors.initials: B
authors.lastname: Fantin
authors.affiliation: Department 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.
authors.forename: M Pineton
authors.initials: MP
authors.lastname: de Chambrun
authors.affiliation: Department 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 ; Université Pierre et Marie Curie-Paris 6, Paris, F-75013 France; AP-HP, Hôpital Pitié-Salpêtrière, Service de Médecine Interne et d'Immunologie clinique, F-75013 Paris, France.
authors.forename: Patrice
authors.initials: P
authors.lastname: Cacoub
authors.affiliation: Department of Hepatology; Hôpital Beaujon, INSERM U773, Service d'hépatologie, 100 boulevard du Général Leclerc, 92118 Clichy cedex, France.
authors.forename: Dominique
authors.initials: D
authors.lastname: Valla
authors.affiliation: Department 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 ; Université Pierre et Marie Curie-Paris 6, Paris, F-75013 France; AP-HP, Hôpital Pitié-Salpêtrière, Service de Médecine Interne et d'Immunologie clinique, F-75013 Paris, France.
authors.forename: David
authors.initials: D
authors.lastname: Saadoun
authors.affiliation: Department of Hepatology; Hôpital Beaujon, INSERM U773, Service d'hépatologie, 100 boulevard du Général Leclerc, 92118 Clichy cedex, France.
authors.forename: Aurélie
authors.initials: A
authors.lastname: Plessier
mesh_terms: D000293:Adolescent; D000328:Adult; D001528:Behcet Syndrome / Q000150:complications* / Q000188:drug therapy; D006502:Budd-Chiari Syndrome / Q000150:complications* / Q000188:drug therapy; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D012307:Risk Factors; D055815:Young Adult
keywords: Behcet’s disease; Budd-chiari syndrome; Hepatic vein thrombosis; Immunousuppressive agents