Background/Purpose: Budd-Chiari syndrome (BCS), or hepatic venous outflow obstruction, is a rare and serious complication of Behçet’s disease (BD). The aim of this study was to determine the demographic, clinical, laboratory and management characteristics along with the clinical course of BCS associated with BD.

Methods: Forty-four patients with BD with BCS (31 male, 13 female) were identified in 15 rheumatology centers (Group I). A total of 82 consecutive patients (51 male, 31 female) with BD who did not have clinically apparent BCS during the follow-up were evaluated as the control group (Group II). The demographic, clinical, laboratory, management characteristics, and clinical course of these two groups of patients were recorded according to predefined protocol and compared.

Results: Comparison of the demographic and clinical findings between the Group I and the Group II were as follows: Male gender was more frequent in both group I and group II but, there was no significant difference between the two groups. The mean age of disease onset was 23.1 +/- 6.7 years vs. 27.2 +/- 8.3 years (p=0.04), mean age at diagnosis was 26.3 +/- 6.9 vs. 31 +/- 8.2 (p<0.01), arthritis was 4.5% vs. 28.9% (p=0.001) and cardiac involvement was found to be 15.9% vs. 3.6% (p=0.017). Skin pathergy test positivity was observed in 40.9% vs. 21.7% of the patients (p=0.02), superficial thrombophlebitis was 25% vs. 7.2% (p=0.006), and deep vein thrombosis was 47.7% vs. 14.5% (p<0.01). The frequency of MTHFR mutation in BD patients with BCS was 25%. On diagnosis 46.7% of BD patients with BCS were classified as Child-Pugh A. Serum albumin level was lower (3.3 +/- 0.6 vs. 4.3 +/- 0.5, p<0.001) and serum AST, ALT and ALP levels were higher, and serum INR level was higher (1.4+/-0.5 vs. 1+/-0.2, p=0.001) in patients with BCS. Inferior vena cava obstruction was observed in 56.7% and portal vein thrombosis was seen in 6.7% of the patients with BCS. Mortality in BCS patients with BD was 18.2%. BCS related treatment after diagnosis in patients with BD were as follows: 67.4% of patients were treated with monthly cyclophosphamide intravenous pulses, 52.3% received intravenous pulse corticosteroids, 56.8% used azathioprine, 45.5% had warfarine treatment and 47.7% were treated with low molecular weight heparin. 

Conclusion: This study shows a higher frequency of cardiac involvement, skin pathergy test positivity, superficial thrombophlebitis and 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. BCS still remains as an important mortality cause in BD patients.

« Back to 2015 ACR/ARHP Annual Meeting

ACR Meeting Abstracts - https://acrabstracts.org/abstract/budd-chiari-syndrome-in-behcets-diseasea-retrospective-multicenter-study/