Evolving Trends in Female to Male Incidence and Male Mortality of Primary Biliary Cholangitis

Overview

Journal Sci Rep

Specialty Science

Date 2016 May 20

PMID 27192935

Citations 60

Authors

Ana Lleo

Peter Jepsen

Emanuela Morenghi

Marco Carbone

Luca Moroni

Pier Maria Battezzati

Mauro Podda

Ian R Mackay

M Eric Gershwin

Pietro Invernizzi

Affiliations

Soon will be listed here.

Abstract

Primary biliary cholangitis (PBC) has been regarded as female-predominant without evidence of gender difference in survival. We aimed to compare the overall survival, incidence and prevalence of PBC in two well defined population-based studies over a recent decade, considering also sex ratios and mortality. We have taken advantage of population-wide records, during 2000-2009, in Lombardia, Northern Italy, and Denmark. We focused on the incident cases of PBC, including gender and outcome, among 9.7 million inhabitants of Lombardia and 5.5 million of Denmark. In Lombardia there were 2,970 PBC cases with a female:male ratio of 2.3:1. The age/sex-adjusted annual incidence of PBC was 16.7 per million. Point prevalence was 160 per million on January 1(st) 2009. In Denmark there were 722 cases of incident PBC, female:male ratio was 4.2:1, and the annual incidence was 11.4 per million, a point prevalence of 115 per million in 2009. Cox regression multivariate analysis identified male sex as an independent predictor of all-cause mortality in both Italian (HR 2.36) and Danish population (HR 3.04). Our data indicate for PBC a sex ratio significantly lower than previously cited, a reversal of the usual latitudinal difference in prevalence and a surprisingly higher overall mortality for male patients.

Citing Articles

Current Treatment Regimens and Promising Molecular Therapies for Chronic Hepatobiliary Diseases.

Durazzo M, Ferro A, Navarro-Tableros V, Gaido A, Fornengo P, Altruda F Biomolecules. 2025; 15(1).

PMID: 39858515 PMC: 11763965. DOI: 10.3390/biom15010121.

Mortality in Autoimmune Liver Disease in Sweden: A Population-Based Cohort Study of 9,654 Patients.

Lasyte I, Widman L, Bergquist A, Hagstrom H Liver Int. 2025; 45(2):e70007.

PMID: 39840802 PMC: 11752691. DOI: 10.1111/liv.70007.

Molecular Mechanisms of Fibrosis in Cholestatic Liver Diseases and Regenerative Medicine-Based Therapies.

Wang W, Lian H, Liang Y, Ye Y, Tam P, Chen Y Cells. 2024; 13(23).

PMID: 39682745 PMC: 11640075. DOI: 10.3390/cells13231997.

Current Landscape and Evolving Therapies for Primary Biliary Cholangitis.

Fiorucci S, Urbani G, Di Giorgio C, Biagioli M, Distrutti E Cells. 2024; 13(18.

PMID: 39329760 PMC: 11429758. DOI: 10.3390/cells13181580.

Evolutionary relationship between antimitochondrial antibody positivity and primary biliary cholangitis in Taiwan: a 16-year hospital cohort study.

Chang M, Cheng J, Le P, Chen W, Ku H, Chien R Therap Adv Gastroenterol. 2024; 17:17562848241241227.

PMID: 38560427 PMC: 10981211. DOI: 10.1177/17562848241241227.

References

Sakauchi F, Mori M, Zeniya M, Toda G . A cross-sectional study of primary biliary cirrhosis in Japan: utilization of clinical data when patients applied to receive public financial aid. J Epidemiol. 2005; 15(1):24-8. PMC: 7817373. DOI: 10.2188/jea.15.24. View

Lofgren J, Jarnerot G, Danielsson D, Hemdal I . Incidence and prevalence of primary biliary cirrhosis in a defined population in Sweden. Scand J Gastroenterol. 1985; 20(5):647-50. DOI: 10.3109/00365528509089711. View

Beuers U, Gershwin M, Gish R, Invernizzi P, Jones D, Lindor K . Changing nomenclature for PBC: From 'cirrhosis' to 'cholangitis'. Clin Res Hepatol Gastroenterol. 2015; 39(5):e57-9. DOI: 10.1016/j.clinre.2015.08.001. View

Cordell H, Han Y, Mells G, Li Y, Hirschfield G, Greene C . International genome-wide meta-analysis identifies new primary biliary cirrhosis risk loci and targetable pathogenic pathways. Nat Commun. 2015; 6:8019. PMC: 4580981. DOI: 10.1038/ncomms9019. View

Kim W, Lindor K, Locke 3rd G, Therneau T, Homburger H, Batts K . Epidemiology and natural history of primary biliary cirrhosis in a US community. Gastroenterology. 2000; 119(6):1631-6. DOI: 10.1053/gast.2000.20197. View

Invernizzi P . Geoepidemiology of autoimmune liver diseases. J Autoimmun. 2009; 34(3):J300-6. DOI: 10.1016/j.jaut.2009.12.002. View

Andersen T, Madsen M, Jorgensen J, Mellemkjoer L, Olsen J . The Danish National Hospital Register. A valuable source of data for modern health sciences. Dan Med Bull. 1999; 46(3):263-8. View

Wang Y, Yang W, Yang J, Jia Y, Tang W, Gershwin M . Systems biologic analysis of T regulatory cells genetic pathways in murine primary biliary cirrhosis. J Autoimmun. 2015; 59:26-37. PMC: 4829434. DOI: 10.1016/j.jaut.2015.01.011. View

Boonstra K, Beuers U, Ponsioen C . Epidemiology of primary sclerosing cholangitis and primary biliary cirrhosis: a systematic review. J Hepatol. 2012; 56(5):1181-1188. DOI: 10.1016/j.jhep.2011.10.025. View

10.

Invernizzi P, Miozzo M, Battezzati P, Bianchi I, Grati F, Simoni G . Frequency of monosomy X in women with primary biliary cirrhosis. Lancet. 2004; 363(9408):533-5. DOI: 10.1016/S0140-6736(04)15541-4. View

11.

Beuers U, Gershwin M, Gish R, Invernizzi P, Jones D, Lindor K . Changing nomenclature for PBC: from 'cirrhosis' to 'cholangitis'. Gastroenterology. 2015; 149(6):1627-9. DOI: 10.1053/j.gastro.2015.08.031. View

12.

Katsumi T, Tomita K, Leung P, Yang G, Gershwin M, Ueno Y . Animal models of primary biliary cirrhosis. Clin Rev Allergy Immunol. 2015; 48(2-3):142-53. DOI: 10.1007/s12016-015-8482-y. View

13.

Beuers U, Gershwin M, Gish R, Invernizzi P, Jones D, Lindor K . Changing nomenclature for PBC: From 'cirrhosis' to 'cholangitis'. Hepatology. 2015; 62(5):1620-2. DOI: 10.1002/hep.28140. View

14.

Sood S, Gow P, Christie J, Angus P . Epidemiology of primary biliary cirrhosis in Victoria, Australia: high prevalence in migrant populations. Gastroenterology. 2004; 127(2):470-5. DOI: 10.1053/j.gastro.2004.04.064. View

15.

Beuers U, Gershwin M, Gish R, Invernizzi P, Jones D, Lindor K . Changing nomenclature for PBC: from 'cirrhosis' to 'cholangitis'. Gut. 2015; 64(11):1671-2. DOI: 10.1136/gutjnl-2015-310593. View

16.

Zoller B, Li X, Sundquist J, Sundquist K . Risk of pulmonary embolism in patients with autoimmune disorders: a nationwide follow-up study from Sweden. Lancet. 2011; 379(9812):244-9. DOI: 10.1016/S0140-6736(11)61306-8. View

17.

Prince M, Chetwynd A, Diggle P, Jarner M, Metcalf J, James O . The geographical distribution of primary biliary cirrhosis in a well-defined cohort. Hepatology. 2001; 34(6):1083-8. DOI: 10.1053/jhep.2001.29760. View

18.

Kaplan M, Gershwin M . Primary biliary cirrhosis. N Engl J Med. 2005; 353(12):1261-73. DOI: 10.1056/NEJMra043898. View

19.

Trivedi P, Lammers W, van Buuren H, Pares A, Floreani A, Janssen H . Stratification of hepatocellular carcinoma risk in primary biliary cirrhosis: a multicentre international study. Gut. 2015; 65(2):321-9. DOI: 10.1136/gutjnl-2014-308351. View

20.

Guillemin F . Describing the epidemiology of rheumatic diseases: methodological aspects. Curr Opin Rheumatol. 2012; 24(2):187-92. DOI: 10.1097/BOR.0b013e32834ff314. View