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Prognostic Factors and Survival Analysis of Antimitochondrial Antibody-Positive Primary Biliary Cirrhosis in Chinese Patients

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Abstract

Background and Aims

Primary biliary cirrhosis (PBC) is a relatively uncommon liver disease, and information on the prognosis and survival of PBC patients in mainland China is lacking. We therefore conducted a retrospective study to investigate the prognostic factors and survival in Chinese PBC patients.

Methods

Between October 2001 and May 2009, patients registered at Beijing You’an Hospital with abnormal liver function and serum positivity for antimitochondrial antibody (AMA) and/or AMA-M2 (n = 391) were screened. Patients diagnosed with PBC were identified, and their medical data were reviewed and analyzed for mortality predictors.

Results

A total of 147 PBC patients were identified (mean age: 54 years, range: 28–81), of whom 126 (85.7%) were women. At the time of diagnosis, 119 patients (81.0%) were symptomatic, 28(19.0%) had hepatic decompensation, and no patients were asymptomatic. During a median follow-up period of 48 months (range: 2–312), 36 patients (24.5%) died or underwent liver transplantation, and 65 patients (44.2%) developed hepatic decompensation. The overall 5-year survival rate was 79%. Multivariate analysis indicated that Mayo risk score ≥6.11(P = 0.008), and serum IgG ≥ 17.20 g/l (P = 0.016) were associated with mortality.

Conclusions

Most Chinese PBC patients in this study were symptomatic at diagnosis and had significant mortality. Mayo risk score, and serum IgG were independent prognostic factors for survival.

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References

  1. Lindor KD, Gershwin ME, Poupon R, Kaplan M, Bergasa NV, Heathcote EJ. American association for study of liver diseases. Primary biliary cirrhosis. Hepatology. 2009;50:291–308.

    Article  PubMed  Google Scholar 

  2. Gershwin ME, Mackay IR, Sturgess A, Coppel RL. Identification and specificity of a cDNA encoding the 70 kd mitochondrial antigen recognized in primary biliary cirrhosis. J Immunol. 1987;138:3525–3531.

    PubMed  CAS  Google Scholar 

  3. Wong GL, Hui AY, Wong VW, Chan FK, Sung JJ, Chan HL. A retrospective study on clinical features and prognostic factors of biopsy-proven primary biliary cirrhosis in Chinese patients. Am J Gastroenterol. 2005;100:2205–2211.

    Article  PubMed  Google Scholar 

  4. Su CW, Hung HH, Huo TI, et al. Natural history and prognostic factors of primary biliary cirrhosis in Taiwan: A follow-up study up to 18 years. Liver Int. 2008;28:1305–1313.

    Article  PubMed  CAS  Google Scholar 

  5. Wong RK, Lim SG, Wee A, Chan YH, Aung MO, Wai CT. 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.

    Article  PubMed  CAS  Google Scholar 

  6. Dickson ER, Grambsch PM, Fleming TR, Fisher LD, Langworthy A. Prognosis in primary biliary cirrhosis: Model for decision making. Hepatology. 1989;10:1–7.

    Article  PubMed  CAS  Google Scholar 

  7. Prince M, Chetwynd A, Newman W, Metcalf JV, James OF. Survival and symptom progression in a geographically based cohort of patients with primary biliary cirrhosis: Follow-up for up to 28 years. Gastroenterology. 2002;123:1044–1051.

    Article  PubMed  Google Scholar 

  8. Parés A, Caballería L, Rodés J. Excellent long-term survival in patients with primary biliary cirrhosis and biochemical response to ursodeoxycholic acid. Gastroenterology. 2006;130:715–720.

    Article  PubMed  Google Scholar 

  9. Peters MG, Di Bisceglie AM, Kowdley KV, et al. Differences between Caucasian, African American, and Hispanic patients with primary biliary cirrhosis in the United States. Hepatology. 2007;46:769–775.

    Article  PubMed  Google Scholar 

  10. Christensen E. Prognostic models including the Child-Pugh, MELD and Mayo risk scores—Where are we and where should we go? J Hepatol. 2004;41:344–350.

    Article  PubMed  Google Scholar 

  11. European Association for the Study of the Liver. EASL clinical practice guidelines: Management of cholestatic liver diseases. J Hepatol. 2009;51:237–267.

    Article  Google Scholar 

  12. Invernizzi P, Crosignani A, Battezzati PM, et al. Comparison of the clinical features and clinical course of antimitochondrial antibody-positive and -negative primary biliary cirrhosis. Hepatology. 1997;25:1090–1095.

    Article  PubMed  CAS  Google Scholar 

  13. Ludwig J, Dickson ER, McDonald GS. Staging of chronic nonsuppurative destructive cholangitis (syndrome of primary biliary cirrhosis). Virchows Arch A Pathol Anat Histol. 1978;379:103–112.

    Article  PubMed  CAS  Google Scholar 

  14. Velázquez RF, Rodríguez M, Navascués CA, et al. Prospective analysis of risk factors for hepatocellular carcinoma in patients with liver cirrhosis. Hepatology. 2003;37:520–527.

    Article  PubMed  Google Scholar 

  15. Cox DR. Regression models and life tables. J R Stat Soc B. 1972;34:187–220.

    Google Scholar 

  16. Liu B, Shi XH, Zhang FC, Zhang W, Gao LX. Antimitochondrial antibody-negative primary biliary cirrhosis: A subset of primary biliary cirrhosis. Liver Int. 2008;28:233–239.

    Article  PubMed  Google Scholar 

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

    Article  PubMed  Google Scholar 

  18. Kim WR, Lindor KD, Locke GR 3rd, et al. Epidemiology and natural history of primary biliary cirrhosis in a US community. Gastroenterology. 2000;119:1631–1636.

    Article  PubMed  CAS  Google Scholar 

  19. Yan HP, Zhuang H, Liu YM, et al. Immunological characteristics of primary biliary cirrhosis patients. Zhonghua Gan Zang Bing Za Zhi. 2005;13:12–16.

    PubMed  CAS  Google Scholar 

  20. Gu EL, Yao GB. The clinical characteristics of primary biliary cirrhosis in China: a systematic review. Zhonghua Gan Zang Bing Za Zhi. 2009;17:861–866.

    PubMed  Google Scholar 

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

    Article  PubMed  Google Scholar 

  22. Selmi C, Lleo A, Invernizzi P, Gershwin ME. Part III: Autoimmune liver diseases. Chapter 18: Primary biliary cirrhosis and autoimmune cholangitis. In: Gershwin ME, Jone MV, Michael PM, eds. Liver immunology: Principles and practice. Totowa, New Jersey: Humana; 2007:235–247.

    Chapter  Google Scholar 

  23. Inoue K, Hirohara J, Nakano T, et al. Prediction of prognosis of primary biliary cirrhosis in Japan. Liver. 1995;15:70–77.

    PubMed  CAS  Google Scholar 

  24. Kaplan MM, Gershwin ME. Primary biliary cirrhosis. N Engl J Med. 2005;353:1261–1273.

    Article  PubMed  CAS  Google Scholar 

  25. Prince MI, Chetwynd A, Craig WL, Metcalf JV, James OF. Asymptomatic primary biliary cirrhosis: clinical features, prognosis, and symptom progression in a large population based cohort. Gut. 2004;53:865–870.

    Article  PubMed  CAS  Google Scholar 

  26. Gershwin ME, Selmi C, Worman HJ, et al. Risk factors and comorbidities in primary biliary cirrhosis: A controlled interview-based study of 1032 patients. Hepatology. 2005;42:1194–1202.

    Article  PubMed  Google Scholar 

  27. Yang W, Lu J, Weng J, et al. Prevalence of diabetes among men and women in China. N Engl J Med. 2010;362:1090–1101.

    Article  PubMed  CAS  Google Scholar 

  28. Hano T. Pathohistological study on liver cirrhosis in diabetes mellitus. Kobe J Med Sci. 1968;14:87–106.

    PubMed  CAS  Google Scholar 

  29. Borgaonkar MR, Morgan DG. Primary biliary cirrhosis and type II autoimmune polyglandular syndrome. Can J Gastroenterol. 1999;13:767–770.

    PubMed  CAS  Google Scholar 

  30. Invernizzi P, Selmi C, Ranftler C, Podda M, Wesierska-Gadek J. Antinuclear antibodies in primary biliary cirrhosis. Semin Liver Dis. 2005;25:298–310.

    Article  PubMed  Google Scholar 

  31. Nakamura M, Shimizu-Yoshida Y, Takii Y, et al. Antibody titer to gp210-C terminal peptide as a clinical parameter for monitoring primary biliary cirrhosis. J Hepatol. 2005;42:386–392.

    Article  PubMed  CAS  Google Scholar 

  32. Nakamura M, Komori A, Ito M, et al. Predictive role of anti-gp210 and anticentromere antibodies in long-term outcome of primary biliary cirrhosis. Hepatol Res. 2007;37:s412–s419.

    Article  PubMed  CAS  Google Scholar 

  33. Worman HJ. Nuclear envelope protein autoantigens in primary biliary cirrhosis. Hepatol Res. 2007;37:S406–S411.

    Article  PubMed  CAS  Google Scholar 

  34. Liang XF, Chen YS, Wang XJ, et al. A study on the sero-epidemiology of hepatitis B in Chinese population aged over 3-years old. Zhonghua Liu Xing Bing Xue Za Zhi. 2005;26:655–658.

    PubMed  Google Scholar 

  35. Chazouilleres O, Wendum D, Serfaty L, Montembault S, Rosmorduc O, Poupon R. Primary biliary cirrhosis-autoimmunehepatitis overlap syndrome: Clinical features and response to therapy. Hepatology. 1998;28:296–301.

    Article  PubMed  CAS  Google Scholar 

Download references

Acknowledgments

This work was supported by National Natural Science Foundation of China (30571723), Beijing Natural Science Foundation (7092046), National “11.5” Key Program for Infectious Diseases (2009ZX10004-309) and Youth Foundation of Beijing Municipal Bureau of Health (QN 2008-032).

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Correspondence to Hui-Ping Yan.

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Zhao, DT., Liao, HY., Liu, YM. et al. Prognostic Factors and Survival Analysis of Antimitochondrial Antibody-Positive Primary Biliary Cirrhosis in Chinese Patients. Dig Dis Sci 56, 2750–2757 (2011). https://doi.org/10.1007/s10620-011-1661-7

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  • DOI: https://doi.org/10.1007/s10620-011-1661-7

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