Budd-Chiari syndrome: epidemiological and clinical characteristics of a case series in Northwest Spain

Abstract

Introduction: Budd-Chiari syndrome (BCS) is a rare vascular disease of the liver, characterised by occlusion of the venous outflow tract. Cancer, pyogenic liver infection, and prothrombotic haematological conditions are the most frequent causes of BCS. The treatment and prognosis of the disease are closely related to the underlying cause. Methods: This is a retrospective case-series study performed in Spain, in a health area of around 523,000 inhabitants. Cases were identified in the discharge database of the hospital between 2000 and 2020. Epidemiological, clinical, therapeutic, and prognosis data were obtained from the patient medical records. Results: A total of 15 cases were identified. Most of them were male patients (n = 8, 53.3%) with a median age of 52 years. The most common cause of BCS was cancer (n = 6, 40.0%) followed by liver abscesses (n = 4, 26.7%). The most frequent clinical course was subacute hepatitis (n = 8, 53.3%); 12 of the 15 patients (80%) received anticoagulant treatment, and interventional treatment was carried out in 4 patients (26.7%). Seven patients died within 6 months (46.7%), 6 of them due to progression of the underlying disease, most often cancer; 2 patients (13.3%) developed liver cirrhosis after BCS. Discussion: The incidence of BCS was low but higher than in other European studies. In addition, this current research showed a different aetiology than previously described. The mortality rate was extremely high and closely related to the underlying disease. The involvement of classic prothrombotic haematological factors was less common than previously described.