Budd-Chiari syndrome epidemiology and demographics

Editor-In-Chief: C. Michael Gibson, M.S., M.D. [1]; Associate Editor(s)-in-Chief: Mazia Fatima, MBBS [2]

Overview

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.

Epidemiology and Demographics

The epidemiology and demographics of Budd-Chiari syndrome are as follows:^[1]^[2]

Prevalence

In 2009-2013, the prevalence of Budd-Chiari syndrome was estimated to be 8 cases per 100,000 individuals in South Korea.

Incidence

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.

Case Fatality Rate

The annual case fatality rate of Budd-Chiari syndrome is approximately 2800 per 100,000 individuals.

Age

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.

Gender

Females are more commonly affected with Budd-Chiari syndrome than males. The female-to-male ratio is approximately 1.8 to 1.

Developed Countries

Budd-Chiari syndrome in the United States is predominantly seen in women and is commonly associated with hematologic disorders.

Developing Countries

Congenital membranous forms is the most common cause of Budd-Chiari syndrome worldwide and particularly in Asia.

References

↑ Ki M, Choi HY, Kim KA, Kim BH, Jang ES, Jeong SH (2016). “Incidence, prevalence and complications of Budd-Chiari syndrome in South Korea: a nationwide, population-based study”. Liver Int. 36 (7): 1067–73. doi:10.1111/liv.13008. PMID 26558363.
↑ Rajani R, Melin T, Björnsson E, Broomé U, Sangfelt P, Danielsson A, Gustavsson A, Grip O, Svensson H, Lööf L, Wallerstedt S, Almer SH (2009). “Budd-Chiari syndrome in Sweden: epidemiology, clinical characteristics and survival – an 18-year experience”. Liver Int. 29 (2): 253–9. doi:10.1111/j.1478-3231.2008.01838.x. PMID 18694401.

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[pmid26558363-1] Ki M, Choi HY, Kim KA, Kim BH, Jang ES, Jeong SH (2016). “Incidence, prevalence and complications of Budd-Chiari syndrome in South Korea: a nationwide, population-based study”. Liver Int. 36 (7): 1067–73. doi:10.1111/liv.13008. PMID 26558363.

[pmid18694401-2] Rajani R, Melin T, Björnsson E, Broomé U, Sangfelt P, Danielsson A, Gustavsson A, Grip O, Svensson H, Lööf L, Wallerstedt S, Almer SH (2009). “Budd-Chiari syndrome in Sweden: epidemiology, clinical characteristics and survival – an 18-year experience”. Liver Int. 29 (2): 253–9. doi:10.1111/j.1478-3231.2008.01838.x. PMID 18694401.

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