Epidemiology of Budd-Chiari syndrome: A systematic review and meta-analysis

Background and aims: The global epidemiological data of Budd-Chiari syndrome (BCS) are scant. A systemic review and meta-analysis aimed to estimate the incidence and prevalence of BCS.

Methods: PubMed, EMBASE, and Cochrane Library databases were searched. The Newcastle-Ottawa Scale was used to assess the study quality. The pooled incidence and prevalence of BCS with 95% confidence intervals (CIs) were calculated by using a random-effect model. The heterogeneity was assessed by the Cochran's Q-test and I² statistics. Subgroup analysis was conducted based on the study region (Asia or Europe).

Results: Overall, six studies were included. Among them, 2 studies were performed in Asian countries (i.e., Japan and South Korea) and 4 in European countries (i.e., Denmark, Sweden, Italy, and France). All of them were of high quality. The annual incidence of BCS was 0.168-4.09 per million. The prevalence of BCS was 2.40-33.10 per million. Meta-analyses showed that the pooled annual incidence of BCS was 1 per million (95% CI = 0.225-3 per million) and the pooled prevalence of BCS was 11 per million (95% CI = 4-21 per million). The heterogeneity among studies was statistically significant. Subgroup meta-analyses demonstrated that the pooled annual incidence of BCS was 0.469 per million in Asia and 2 per million in Europe and the pooled prevalence of BCS was 5 per million in Asia.

Conclusion: Evidence from meta-analyses of existing literature confirmed that BCS should be a rare vascular liver disease. BCS may not be more common in Asia than Europe. More epidemiological data in other countries should be warranted.