In 2009-2013, the prevalence of Budd-Chiari syndrome was estimated to be 8 patients cases per 100,000 individuals in South Korea. Budd-Chiari syndrome is a rare disease. The annual incidence of Budd-Chiari syndrome was estimated to be about 11 cases per 100,000 individuals in Sweden. The annual case fatality rate of Budd-Chiari syndrome is approximately 2800 per 100,000 patients. Budd-Chiari syndrome commonly presents in the third or fourth decade of life. The median age at diagnosis is 51 years. Budd-Chiari syndrome is rare in children. Females are more commonly affected with Budd-Chiari syndrome than males. The female-to-male ratio is approximately 1.8  to 1. Budd-Chiari syndrome in the United States is predominantly seen in women and is commonly associated with hematologic disorders. Congenital membranous form is the most common cause of Budd-Chiari syndrome worldwide and particularly in Asia.
Budd-Chiari syndrome epidemiology and demographics
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