Skip to main content

Advertisement

SpringerLink
Log in

Epidemiology and clinical course of primary biliary cholangitis in the Asia–Pacific region: a systematic review and meta-analysis

  • Original Article
  • Published:
Hepatology International Aims and scope Submit manuscript

Abstract

Aims

Epidemiological studies on primary biliary cholangitis (PBC) show heterogeneity. The aim of the present study was to synthesize the prevalence, incidence and clinical course of PBC in the Asia–Pacific region.

Methods

PubMed, Medline, Cochrane library and EMBASE were searched for epidemiology and clinical course of PBC published up to July, 2019. Meta-analysis was conducted on the epidemiology and clinical course (decompensation, hepatocellular carcinoma and death/liver transplantation) of PBC patients. Random-effect model and fixed-effect model were used to evaluate the pooled prevalence, incidence, mortality/liver transplantation and their 95% confidence intervals as appropriate. Subgroup analysis was performed by stratification with gender, pre- and post-UDCA era, sub-region and publication year. Meta-regression was used to examine the heterogeneity.

Results

Out of 3460 studies, 18 studies from 7 countries/regions were finally included. The overall prevalence of PBC was 118.75 cases per million (95% CI 49.96–187.55) in the Asia–Pacific region, with the high, medium and low prevalence being in Japan and China (191.18 cases per million), New Zealand (99.16 cases per million) and South Korea and Australia (39.09 cases per million), respectively. The incidence of PBC was 8.55 cases per million per year (95% CI 8.05–9.06). The 5-year accumulative incidence of decompensation, HCC and death/liver transplantation in PBC patients was 6.95% (95% CI 2.07–11.83%), 1.54% (95% CI 0.9–2.19%) and 4.02% (95% CI 2.49–5.54%), respectively.

Conclusion

In the Asia–Pacific region, the prevalence and incidence of PBC are higher than once expected. PBC tends to be diagnosed at older age and has a relatively low incidence of HCC in this region.

Graphic abstract

This is a preview of subscription content, log in via an institution to check access.

Access this article

Log in via an institution

Price excludes VAT (USA)
Tax calculation will be finalised during checkout.

Instant access to the full article PDF.

Institutional subscriptions

Fig. 1
Fig. 2
Fig. 3
Fig. 4
Fig. 5
Fig. 6
Fig. 7

Similar content being viewed by others

References

  1. Tanaka A, Leung PSC, Gershwin ME. Evolution of our understanding of PBC. Best Pract Res Clin Gastroenterol 2018;34–35:3–9

    PubMed  Google Scholar 

  2. Lindor KD, Gershwin ME, Poupon R, et al. Primary biliary cirrhosis. Hepatology 2009;50:291–308

    PubMed  Google Scholar 

  3. Lleo A, Marzorati S, Anaya JM, et al. Primary biliary cholangitis: a comprehensive overview. Hepatol Int 2017;11:485–499

    PubMed  Google Scholar 

  4. Tanaka A, Takikawa H, Mochida S, et al. Changing nomenclature for PBC from “Primary Biliary Cirrhosis” to “Primary Biliary Cholangitis”. Nihon Shokakibyo Gakkai Zasshi 2016;113:1165–1167

    PubMed  Google Scholar 

  5. Investigators LV. Epidemiology, practice of ventilation and outcome for patients at increased risk of postoperative pulmonary complications: LAS VEGAS—an observational study in 29 countries. Eur J Anaesthesiol 2017;34:492–507

    Google Scholar 

  6. Carey EJ, Ali AH, Lindor KD. Primary biliary cirrhosis. Lancet 2015;386:1565–1575

    CAS  PubMed  Google Scholar 

  7. Lu M, Li J, Haller IV, et al. Factors Associated With Prevalence and Treatment of Primary Biliary Cholangitis in United States Health Systems. Clin Gastroenterol Hepatol 2018;16(1333–1341):e6

    Google Scholar 

  8. Boonstra K, Kunst AE, Stadhouders PH, et al. Rising incidence and prevalence of primary biliary cirrhosis: a large population-based study. Liver Int 2014;34:e31–e38

    PubMed  Google Scholar 

  9. Myers RP, Shaheen AA, Fong A, et al. Epidemiology and natural history of primary biliary cirrhosis in a Canadian health region: a population-based study. Hepatology 2009;50:1884–1892

    PubMed  Google Scholar 

  10. Yoshida EM, Mason A, Peltekian KM, et al. Epidemiology and liver transplantation burden of primary biliary cholangitis: a retrospective cohort study. CMAJ Open 2018;6:E664–E670

    PubMed  PubMed Central  Google Scholar 

  11. Boonstra K, Beuers U, Ponsioen CY. Epidemiology of primary sclerosing cholangitis and primary biliary cirrhosis: a systematic review. J Hepatol 2012;56:1181–1188

    PubMed  Google Scholar 

  12. Kim KA, Ki M, Choi HY, et al. Population-based epidemiology of primary biliary cirrhosis in South Korea. Aliment Pharmacol Ther 2016;43:154–162

    PubMed  Google Scholar 

  13. Chen S, Duan W, Li M et al. Prognosis of 732 ursodeoxycholic acid-treated patients with primary biliary cholangitis: a single center follow-up study from China. J Gastroenterol Hepatol 2018

  14. Duan W, Ou X, Wang X, et al. Efficacy and safety of fenofibrate add-on therapy for patients with primary biliary cholangitis and a suboptimal response to UDCA. Rev Esp Enferm Dig 2018;110:557–563

    PubMed  Google Scholar 

  15. Harada K, Nakanuma Y. Prevalence and risk factors of hepatocellular carcinoma in Japanese patients with primary biliary cirrhosis. Hepatol Res 2014;44:133–140

    PubMed  Google Scholar 

  16. Hu C, Deng C, Song G, et al. Prevalence of autoimmune liver disease related autoantibodies in Chinese patients with primary biliary cirrhosis. Dig Dis Sci 2011;56:3357–3363

    CAS  PubMed  Google Scholar 

  17. Harada K, Hirohara J, Ueno Y, et al. Incidence of and risk factors for hepatocellular carcinoma in primary biliary cirrhosis: national data from Japan. Hepatology 2013;57:1942–1949

    PubMed  Google Scholar 

  18. Sood S, Gow PJ, Christie JM, et al. Epidemiology of primary biliary cirrhosis in Victoria, Australia: high prevalence in migrant populations. Gastroenterology 2004;127:470–475

    PubMed  Google Scholar 

  19. Liberati A, Altman DG, Tetzlaff J, et al. The PRISMA statement for reporting systematic reviews and meta-analyses of studies that evaluate healthcare interventions: explanation and elaboration. BMJ 2009;339:b2700

    PubMed  PubMed Central  Google Scholar 

  20. Shamliyan T, Kane RL, Dickinson S. A systematic review of tools used to assess the quality of observational studies that examine incidence or prevalence and risk factors for diseases. J Clin Epidemiol 2010;63:1061–1070

    PubMed  Google Scholar 

  21. Shamliyan TA, Kane RL, Ansari MT, et al. Development of quality criteria to evaluate nontherapeutic studies of incidence, prevalence, or risk factors of chronic diseases: pilot study of new checklists. J Clin Epidemiol 2011;64(6):637–657

    PubMed  Google Scholar 

  22. Watson RG, Angus PW, Dewar M, et al. Low prevalence of primary biliary cirrhosis in Victoria, Australia. Melbourne Liver Group. Gut 1995;36:927–930

    CAS  PubMed  Google Scholar 

  23. Tsuji K, Watanabe Y, Van De Water J, et al. Familial primary biliary cirrhosis in Hiroshima. J Autoimmun 1999;13:171–178

    CAS  PubMed  Google Scholar 

  24. Ohba K, Omagari K, Kinoshita H, et al. Primary biliary cirrhosis among atomic bomb survivors in Nagasaki, Japan. J Clin Epidemiol 2001;54:845–850

    CAS  PubMed  Google Scholar 

  25. Sood S, Gow PJ, Christie JM, et al. Epidemiology of primary biliary cirrhosis in Victoria, Australia: high prevalence in migrant populations. Gastroenterology 2004;127:470–475

    PubMed  Google Scholar 

  26. Amarapurkar DN, Patel ND. Spectrum of autoimmune liver diseases in western India. J Gastroenterol Hepatol 2007;22:2112–2117

    PubMed  Google Scholar 

  27. Fung KT, Fung J, Lai CL, et al. Etiologies of chronic liver diseases in Hong Kong. Eur J Gastroenterol Hepatol 2007;19:659–664

    PubMed  Google Scholar 

  28. Wong RK, Lim SG, Wee A, et al. Primary biliary cirrhosis in Singapore: evaluation of demography, prognostic factors and natural course in a multi-ethnic population. J Gastroenterol Hepatol 2008;23:599–605

    CAS  PubMed  Google Scholar 

  29. Azemoto N, Abe M, Murata Y, et al. Clinical profile of primary biliary cirrhosis that was diagnosed as symptomatic primary biliary cirrhosis according to the revised diagnostic criteria in Japan. Intern Med 2010;49:1073–1038

    PubMed  Google Scholar 

  30. Liu H, Liu Y, Wang L, et al. Prevalence of primary biliary cirrhosis in adults referring hospital for annual health check-up in Southern China. BMC Gastroenterol 2010;10:100

    PubMed  PubMed Central  Google Scholar 

  31. Ngu JH, Gearry RB, Wright AJ, et al. Low incidence and prevalence of primary biliary cirrhosis in Canterbury, New Zealand: a population-based study. Hepatol Int 2012;6:796–800

    PubMed  Google Scholar 

  32. Rong G, Wang H, Bowlus CL, et al. Incidence and risk factors for hepatocellular carcinoma in primary biliary cirrhosis. Clin Rev Allergy Immunol 2015;48:132–141

    CAS  PubMed  Google Scholar 

  33. Zhang XX, Wang LF, Jin L, et al. Primary biliary cirrhosis-associated hepatocellular carcinoma in Chinese patients: incidence and risk factors. World J Gastroenterol 2015;21:3554–3563

    CAS  PubMed  PubMed Central  Google Scholar 

  34. Cheung KS, Seto WK, Fung J, et al. Epidemiology and Natural History of Primary Biliary Cholangitis in the Chinese: a Territory-Based Study in Hong Kong between 2000 and 2015. Clin Transl Gastroenterol 2017;8:e116

    PubMed  PubMed Central  Google Scholar 

  35. Fan X, Wang T, Shen Y, et al. Underestimated male prevalence of primary biliary cholangitis in China: results of a 16-yr cohort study involving 769 patients. Sci Rep 2017;7:6560

    PubMed  PubMed Central  Google Scholar 

  36. Tanaka A, Mori M, Matsumoto K, et al. Increase trend in the prevalence and male-to-female ratio of primary biliary cholangitis, autoimmune hepatitis, and primary sclerosing cholangitis in Japan. Hepatol Res 2019

  37. Miyakawa H, Tanaka A, Kikuchi K, et al. Detection of antimitochondrial autoantibodies in immunofluorescent AMA-negative patients with primary biliary cirrhosis using recombinant autoantigens. Hepatology 2001;34:243–248

    CAS  PubMed  Google Scholar 

  38. Floreani A, Mangini C. Primary biliary cholangitis: old and novel therapy. Eur J Intern Med 2018;47:1–5

    CAS  PubMed  Google Scholar 

  39. Lammers WJ, van Buuren HR, Hirschfield GM, et al. Levels of alkaline phosphatase and bilirubin are surrogate end points of outcomes of patients with primary biliary cirrhosis: an international follow-up study. Gastroenterology 2014;147:1338–1349 (quiz e15)

    CAS  PubMed  Google Scholar 

  40. Cavazza A, Caballeria L, Floreani A, et al. Incidence, risk factors, and survival of hepatocellular carcinoma in primary biliary cirrhosis: comparative analysis from two centers. Hepatology 2009;50:1162–1168

    PubMed  Google Scholar 

  41. Trivedi PJ, Lammers WJ, van Buuren HR, et al. Stratification of hepatocellular carcinoma risk in primary biliary cirrhosis: a multicentre international study. Gut 2016;65:321–329

    PubMed  Google Scholar 

  42. Harms MH, Lammers WJ, Thorburn D, et al. Major hepatic complications in ursodeoxycholic acid-treated patients with primary biliary cholangitis: risk factors and time trends in incidence and outcome. Am J Gastroenterol 2018;113:254–264

    PubMed  Google Scholar 

  43. Carbone M, Mells GF, Pells G, et al. Sex and age are determinants of the clinical phenotype of primary biliary cirrhosis and response to ursodeoxycholic acid. Gastroenterology 2013;144:560–569 e7 (quiz e13–4)

    Google Scholar 

  44. Di Bisceglie AM. Hepatitis B and hepatocellular carcinoma. Hepatology 2009;49:S56–S60

    PubMed  Google Scholar 

Download references

Funding

This work was supported by grants from the National Science and Technology Major Special Project for New Drug Development (No. 2018ZX09201016), National Science and Technology Major Special Project for Infectious Diseases (No. 2018ZX10302204), the Digestive Medical Coordinated Development Center of Beijing Hospitals Authority (No. XXX 0104) and the Beijing Hospitals Authority Incubating Program (No. PX2019003).

Author information

Author notes

  1. Na Zeng and Weijia Duan contributed equally to this work.

Authors and Affiliations

  1. Clinical Epidemiology and EBM Unit, Beijing Friendship Hospital, Capital Medical University, Beijing, China

    Na Zeng, Shanshan Wu & Yuanyuan Kong

  2. Liver Research Center, Beijing Friendship Hospital, Capital Medical University, Beijing, China

    Weijia Duan, Sha Chen, Hong Ma, Xiaojuan Ou, Hong You & Jidong Jia

  3. Beijing Key Laboratory of Translational Medicine on Liver Cirrhosis, Beijing, China

    Weijia Duan, Sha Chen, Hong Ma, Xiaojuan Ou, Hong You & Jidong Jia

  4. National Clinical Research Center for Digestive Diseases, Beijing, China

    Na Zeng, Weijia Duan, Sha Chen, Shanshan Wu, Hong Ma, Xiaojuan Ou, Hong You, Yuanyuan Kong & Jidong Jia

Authors
  1. Na Zeng
    View author publications

    You can also search for this author in PubMed Google Scholar

  2. Weijia Duan
    View author publications

    You can also search for this author in PubMed Google Scholar

  3. Sha Chen
    View author publications

    You can also search for this author in PubMed Google Scholar

  4. Shanshan Wu
    View author publications

    You can also search for this author in PubMed Google Scholar

  5. Hong Ma
    View author publications

    You can also search for this author in PubMed Google Scholar

  6. Xiaojuan Ou
    View author publications

    You can also search for this author in PubMed Google Scholar

  7. Hong You
    View author publications

    You can also search for this author in PubMed Google Scholar

  8. Yuanyuan Kong
    View author publications

    You can also search for this author in PubMed Google Scholar

  9. Jidong Jia
    View author publications

    You can also search for this author in PubMed Google Scholar

Contributions

NZ, JDJ and YYK designed the study and drafted the manuscript. NZ, WJD and SC extracted the data. NZ, WJD, HM and XJO assessed the quality of evidence. NA and SSW analyzed the data. YYK, HY and JDJ interpreted the results and finalized the manuscript. All the authors approved the final version of the paper.

Corresponding authors

Correspondence to Yuanyuan Kong or Jidong Jia.

Ethics declarations

Conflict of interest

Na Zeng, Weijia Duan, Sha Chen, Shanshan Wu, Hong Ma, Xiaojuan Ou, Hong You, Yuanyuan Kong and Jidong Jia declare no conflict of interests.

Ethical approval

This article does not contain any study with human participants or animals performed by any of the authors.

Additional information

Publisher's Note

Springer Nature remains neutral with regard to jurisdictional claims in published maps and institutional affiliations.

Electronic supplementary material

Below is the link to the electronic supplementary material.

Supplementary material 1 (DOC 1690 kb)

Rights and permissions

Reprints and permissions

About this article

Check for updates. Verify currency and authenticity via CrossMark

Cite this article

Zeng, N., Duan, W., Chen, S. et al. Epidemiology and clinical course of primary biliary cholangitis in the Asia–Pacific region: a systematic review and meta-analysis. Hepatol Int 13, 788–799 (2019). https://doi.org/10.1007/s12072-019-09984-x

Download citation

  • Received:

  • Accepted:

  • Published:

  • Issue Date:

  • DOI: https://doi.org/10.1007/s12072-019-09984-x

Keywords

Search

Navigation