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Budd-Chiari syndrome in Behcet's disease: A report of two cases
Jun Zhou1, Yu Wang2, Yiming Liu2
1Department of Rheumatology and Clinical Immunology, The First Affiliated Hospital of Sun Yat-sen University, Guangzhou, Guangdong 510080, P.R. China.
Insights
Budd-Chiari syndrome (BCS), a severe venous complication of Behcet's disease (BD), requires increased awareness. Early diagnosis and combined TNF-α inhibitor and cyclophosphamide treatment can lead to favorable outcomes in BCS patients.
Area of Science:
- Vascular Medicine
- Hepatology
- Rheumatology
Background:
- Budd-Chiari syndrome (BCS) is a rare but severe venous complication of Behcet's disease (BD).
- BCS involves obstruction of hepatic venous outflow into the inferior vena cava.
- Awareness of BCS is crucial in regions with high BD prevalence.
Purpose of the Study:
- To present two cases of BCS in male Chinese patients with BD.
- To summarize the clinical features, treatment, and outcomes of BD-BCS.
- To highlight diagnostic challenges and treatment implications for BD-BCS.
Main Methods:
- Case report and retrospective analysis of two male Chinese patients with BD and BCS.
- Comparison of clinical characteristics, treatment, and outcomes with existing literature.
- Summary of features specific to Behcet's disease-associated Budd-Chiari syndrome (BD-BCS).
Main Results:
- Both patients presented with insidious onset, abdominal symptoms, and recurrent aphthous ulcers, delaying diagnosis.
- Both responded well to treatment with TNF-α inhibitors and cyclophosphamide (CYC).
- Patient compliance significantly impacted outcomes: good compliance led to therapy withdrawal, while poor compliance resulted in a poor outcome.
Conclusions:
- Multiple vessel involvement is common in vasculo-BD, and misdiagnosis of BD-BCS can occur if other BD symptoms are overlooked.
- BD-BCS carries a high mortality rate, but appropriate treatment can improve patient outcomes.
- Effective management requires recognizing diverse BD manifestations and ensuring treatment adherence.
Abstract:
Budd-Chiari syndrome (BCS) is a rare but severe venous form of Behcet's disease (BD) that is caused by the obstruction of the venous outflow tract that transports blood from hepatic veins into the inferior vena cava. In countries where BD is prevalent, including the Middle East and Far East, BCS awareness is important. In the present study, two cases of BCS are presented in two male Chinese patients with BD. The clinical characteristics, treatment and outcomes were recorded and compared with previous studies, and the features of BD-BCS were summarized. The clinical characteristics of the two patients documented were similar. Each patient presented with insidious onset, abdominal symptoms and recurrent aphthous ulcers. Accurate diagnosis was delayed as other symptoms of BD were overlooked. Each patient responded well to TNF-α inhibitor treatment in combination with cyclophosphamide (CYC). One patient with good compliance was removed from CYC and corticosteroid therapy. Unfortunately, the other patient with poor compliance faced a poor outcome. It was concluded that multiple vessel lesions in ≥2 sites are common in vasculo-BD and that misdiagnosis may occur if other symptoms of BD are not noticed. BD-BCS is associated with a high mortality rate, but appropriate treatment may result in a favorable outcome.