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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.
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.
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