title: Population-based epidemiology of primary biliary cirrhosis in South Korea.
abstract: BACKGROUND
  As a rare disease, only a few population-based epidemiology studies of primary biliary cirrhosis (PBC) have been reported.
  
  
  OBJECTIVE
  To elucidate the nationwide prevalence, incidence, complications, fatality and direct medical costs of PBC in South Korea.
  
  
  METHODS
  The nationwide Health Insurance Review and Assessment Service claims data and Rare Intractable Disease registration data on PBC, identified with the International Classification of Diseases (ICD) 10 code of K74.3, were obtained from 2009 to 2013. Age- and gender-specific prevalence and incidence rates of PBC were calculated, and data on complications, comorbidities, prescribed drugs, therapeutic procedures and direct medical costs were analysed.
  
  
  RESULTS
  A total of 2824 patients over 20 years old with PBC were identified in 2009-2013 (female-to-male ratio 6.2, median age 57 years old). The average age- and sex-adjusted incidence from 2011 to 2013 was 8.57 per million per year, and the average age- and sex-adjusted prevalence from 2009 to 2013 was 47.50 per million population. About 10% of patients presented with complications such as ascites (10.3%), variceal bleeding (5.8%) and/or hepatocellular carcinoma (HCC) (1.3%). Liver transplantation was undertaken in 71 patients (2.5%) for 5 years. Case fatality was 2.2% and the transplantation-free survival was 95.4% for 5 years.
  
  
  CONCLUSIONS
  This is the first report on the nationwide epidemiology of primary biliary cirrhosis in South Korea, demonstrating lower incidence and prevalence rates than those of Western countries, but a considerable disease burden, representing at least 10% were complicated with decompensated cirrhosis or hepatocellular carcinoma requiring liver transplantation.
authors.affiliation: Department of Internal Medicine, Inje University Ilsan Paik Hospital, Goyang, Korea.
authors.forename: K-A
authors.initials: KA
authors.lastname: Kim
authors.affiliation: Department of Cancer Control and Policy, Graduate School of Cancer Science and Policy, National Cancer Center, Goyang, Korea.
authors.forename: M
authors.initials: M
authors.lastname: Ki
authors.affiliation: Department of Cancer Control and Policy, Graduate School of Cancer Science and Policy, National Cancer Center, Goyang, Korea.
authors.forename: H Y
authors.initials: HY
authors.lastname: Choi
authors.affiliation: Center for Liver Cancer, National Cancer Center, Goyang, Korea.
authors.forename: B H
authors.initials: BH
authors.lastname: Kim
authors.affiliation: Departments of Internal Medicine, Seoul National University Bundang Hospital, Seoul National University College of Medicine, Seongnam, Korea.
authors.forename: E S
authors.initials: ES
authors.lastname: Jang
authors.affiliation: Departments of Internal Medicine, Seoul National University Bundang Hospital, Seoul National University College of Medicine, Seongnam, Korea.
authors.forename: S-H
authors.initials: SH
authors.lastname: Jeong
mesh_terms: D000328:Adult; D000368:Aged; D006528:Carcinoma, Hepatocellular / Q000453:epidemiology; D015897:Comorbidity; D004932:Esophageal and Gastric Varices / Q000453:epidemiology; D005260:Female; D006471:Gastrointestinal Hemorrhage / Q000453:epidemiology; D006801:Humans; D015994:Incidence; D008105:Liver Cirrhosis, Biliary / Q000191:economics* / Q000453:epidemiology* / Q000401:mortality / Q000601:surgery; D008113:Liver Neoplasms / Q000453:epidemiology; D016031:Liver Transplantation / Q000706:statistics & numerical data; D008297:Male; D008875:Middle Aged; D015995:Prevalence; D056910:Republic of Korea / Q000453:epidemiology