THU0329 Budd-chiari syndrome in behÇet's disease: a retrospective multicenter study

Background The aim of this study was to determine the demographic, clinical, laboratory and management characteristics along with the clinical course of Budd-Chiari syndrome (BCS) associated with Behcet9s disease (BD). Methods Sixty patients with BD with BCS (40 male, 20 female) were identified in 23 rheumatology centers (Group I). A total of 169 consecutive patients (100 male, 69 female) with BD who did not have clinically apparent BCS during the follow-up were evaluated as the control group (Group II). Results Comparison of the demographic and clinical findings between the Group I and the Group II were as follows: The mean age of disease onset was 23.1 +/- 6.7 years vs. 26.8±0.6 years (p=0.013), mean age at diagnosis was 27.2±0.9 vs. 30.4±0.6 years (p=0.008), arthritis was 10% vs. 28.4% (p=0.002), papulopustular skin lesion was 48.3% vs 69.2% (p=0.003), central nervous system (CNS) involvement 10% vs. 3% (p=0.03), cardiac involvement was 16.7% vs. 2.4% (p Conclusions This study shows a higher frequency of cardiac and CNS involvement, superficial thrombophlebitis, papulopustular skin lesion, deep vein thrombosis in BD patients with BCS. Arthritis was observed less common in BD patients with BCS. The mean age onset was lower in patients with BCS. Medical treatment with immunosuppressive agents and anticoagulation appears to be the treatment of choice in BD patients with BCS. The majority of the patients with BCS were Child–Pugh class A on diagnosis. The inferior vena cava is frequently involved and, often associated with deep vein thrombosis and cardiac involvement. Disclosure of Interest None declared