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.

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).

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.

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.