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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.

Orphanet Journal of Rare Diseases
|September 13, 2014
PubMed
Summary

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.

Keywords:
Behcet’s diseaseBudd-chiari syndromeHepatic vein thrombosisImmunousuppressive agents

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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.