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. 2018 Dec 21;6(4):E664-E670.
doi: 10.9778/cmajo.20180029. Print 2018 Oct-Dec.

Epidemiology and liver transplantation burden of primary biliary cholangitis: a retrospective cohort study

Eric M Yoshida  1 , Andrew Mason  1 , Kevork M Peltekian  1 , Hemant Shah  1 , Sherri Thiele  1 , Richard Borrelli  1 , Aren Fischer  2
Affiliations

Epidemiology and liver transplantation burden of primary biliary cholangitis: a retrospective cohort study

Eric M Yoshida et al. CMAJ Open. .

Abstract

Background: There is a wealth of data documenting the epidemiology of primary biliary cholangitis (PBC) globally; however, the epidemiology of PBC has not been as well studied in Canada. Our study characterized the Canadian prevalence of PBC and the number of liver transplantations because of PBC.

Methods: For this retrospective cohort study we used national hospital administrative records from the Canadian Institute for Health Information, with the exception of Quebec for the prevalence estimate and Quebec and British Columbia for the transplant analysis. Prevalent patients were identified through a diagnostic code for PBC of the Canadian version of the 10th revision of the International Classification of Diseases. PBC transplant patients were identified from their transplant record. Descriptive statistics were used to summarize the characteristics of the study cohorts.

Results: In 2015, 8680 patients with PBC were identified in Canada, translating to a prevalence of 318 cases per million. Annual prevalence by province varied, ranging from 283 (95% confidence interval [CI] 269-297) cases per million to 465 (95% CI 426-504) cases per million, and the 6-year PBC liver transplantation rate ranged from 3.17 (95% CI 1.27-6.54) to 5.92 (95% CI 3.71-9.08) per million. The Atlantic provinces exhibited the highest PBC prevalence and close to the highest 6-year liver transplantation rate (465 [95% CI 426-504] cases per million and 5.70 [95% CI 426-504, 3.19-9.56] cases per million, respectively). We observed the lowest PBC prevalence (283 [95% CI 269-297] cases per million) and the second lowest 6-year liver transplantation rate in Ontario (3.37 [95% CI 2.47-4.50] cases per million).

Interpretation: The prevalence of PBC that we found in Canada is similar to the prevalence reported in other studies, but our work also indicates geographic variation within this country. Given our finding of geographic clustering of PBC across Canada, we hypothesize that environmental and genetic factors contribute to the pathogenesis of this condition.

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Conflict of interest statement

Competing interests: Eric Yoshida has been an investigator of clinical trials sponsored by Intercept, Gilead, Merck, Janssen, AbbVie, Genfit and Spring Bank. He has received speaker fees from Gilead, Merck, AbbVie, Celgene and Intercept. Andrew Mason reports receiving grants from Intercept, Merck, Gilead, AbbVie, Novartis and GlaxoSmithKline during the conduct of the study and grants from Intercept, Merck, Gilead and AbbVie outside the submitted work. He has received advisory board and speaker fees from Intercept. Kevork Peltekian is a member of the national board of directors of the Canadian Liver Foundation. Hemant Shah has received advisory board and speaker fees from AbbVie, Gilead, Intercept, Lupin and Merck.

Figures

Figure 1
Figure 1
(A) Analysis of wait time for liver transplantation for primary biliary cholangitis, showing the percentage of patients waiting to receive a liver transplant for each month after being listed on the transplant list. (B) Analysis of survival after liver transplantation for primary biliary cholangitis.
See this image and copyright information in PMC

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