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

Yoshida, Eric M.; Mason, Andrew L.; Peltekian, Kevork M.; Shah, Hemant; Thiele, Sherri L.; Borrelli, Richard; Fischer, Aren

doi:10.9778/cmajo.20180029

Cited by 13 publications

(13 citation statements)

References 45 publications

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“…In South Korea, while the incidence of AIH was stable, the prevalence increased gradually from 2009 to 2013 . Rising prevalence of PBC over time has consistently been reported from the Netherlands, northern Italy and Denmark, Hong Kong, Canada, and the USA, and it is a point of debate whether the incidence of PBC is also increasing. In PSC, two systematic reviews concluded that the prevalence as well as the incidence of PSC seem to be increasing .…”

Section: Discussionmentioning

confidence: 99%

“…[4][5][6][7][8] Estimation of prevalence of AILDs is critical to assess the burden of these diseases on public health. A number of epidemiological studies have been undertaken for AIH, [9][10][11][12][13][14][15][16][17][18][19] PBC, 13,[20][21][22][23][24][25][26][27][28][29][30][31][32][33][34][35][36] and PSC [37][38][39][40][41][42][43] worldwide, and have shown that prevalence significantly varies depending on regions and ethnicities. Interestingly, several recent studies have indicated that prevalence of all three AILDs has been increasing over the years.…”

Section: Introductionmentioning

confidence: 99%

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Increase trend in the prevalence and male‐to‐female ratio of primary biliary cholangitis, autoimmune hepatitis, and primary sclerosing cholangitis in Japan

Tanaka

Mori

Matsumoto

et al. 2019

Hepatology Research

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Aim Autoimmune liver diseases (AILD) including autoimmune hepatitis (AIH), primary biliary cholangitis (PBC), and primary sclerosing cholangitis (PSC) impose a significant burden on public health, and it is important to estimate their prevalence. We carried out a nationwide, hospital‐based, epidemiological survey of AIH, PBC, and PSC, and compared the results with those from previous surveys. Method We randomly selected health‐care facilities used in the survey from a list of all facilities in Japan. The selection rate was determined according to a stratification based on the facility characteristics and scale. We sent questionnaires to the selected facilities enquiring about the number and sex of patients with AILD who visited the facility in 2016. An identical survey was undertaken for AIH/PBC in 2004 and for PSC in 2007; we carried out a comparative analysis of these data. Results We selected 1793 departments from health centers all over Japan. Of them, 1078 (60.1%) responded to the questionnaires. The number of reported patients with AIH, PBC, and PSC was 8505, 10 847, and 906, respectively, and point prevalence was 23.9 (95% confidence interval, 23.3–24.5) for AIH (8.7 in 2004), 33.8 (33.0–34.6) for PBC (11.6 in 2004), and 1.80 (1.75–1.85) for PSC (0.95 in 2007) per 100 000 population. Male‐to‐female patient ratio of AILD was 1:4.3 for AIH (1:6.9 in 2004), 1:3.9 for PBC (1:7.1 in 2004), and 1:0.88 for PSC (1:1.4 in 2007). Conclusion The current study indicates an increasing trend of prevalence as well as male‐to‐female patient ratio of AILD in Japan.

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Section: Discussionmentioning

confidence: 99%

Section: Introductionmentioning

confidence: 99%

Increase trend in the prevalence and male‐to‐female ratio of primary biliary cholangitis, autoimmune hepatitis, and primary sclerosing cholangitis in Japan

Tanaka

Mori

Matsumoto

et al. 2019

Hepatology Research

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“…Primary biliary cholangitis (PBC) is an uncommon chronic cholestatic liver disease with an estimated prevalence of 318 per million people in Canada. ( 1 ) The disease is characterized by progressive destruction of interlobular bile ducts and is clinically impactful with an increased risk of progression to advanced biliary fibrosis, end‐stage liver disease, and death. Until recently, ursodeoxycholic acid (UDCA) was the only approved therapy widely accepted to improve clinical outcomes in patients with PBC ( 2,3 ) ; however, approximately 40% of patients have incomplete biochemical response to UDCA and remain at risk of disease progression.…”

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confidence: 99%

Real‐World Effectiveness of Obeticholic Acid in Patients with Primary Biliary Cholangitis

et al. 2020

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Patients with primary biliary cholangitis (PBC) with incomplete response to ursodeoxycholic acid are at risk of disease progression and need additional therapy. Obeticholic acid (OCA) was approved in Canada in May 2017, but its effectiveness in a real‐world setting has not been described. We sought to describe our experience with OCA in a Canadian cohort. OCA‐naive patients treated at two Canadian centers were included. Clinical and biochemical data were collected at OCA initiation and during follow‐up. Primary outcomes were changes in serum alkaline phosphatase (ALP), gamma‐glutamyl transferase (GGT), and total bilirubin (TB) over the duration of therapy. Secondary outcomes were changes in alanine aminotransferase (ALT), aspartate aminotransferase (AST), immunoglobulin M (IgM), platelets, and albumin; and achievement of the primary endpoint of the original phase 3 study that led to OCA approval (A Placebo‐Controlled Trial of Obeticholic Acid in Primary Biliary Cholangitis [POISE]), dose reductions, discontinuations, and tolerability. Repeated‐measures models were used to assess changes in biochemistry over time. Sixty‐four patients were included; 4 carried a diagnosis of overlap with autoimmune hepatitis. Mean age was 54.6 years, median ALP was 250 U/L, TB was 13 µmol/L, platelet count was 225 × 109/L, and 24% had liver stiffness measurements ≥16.9 kPa. There was a significant reduction in mean ALP of 55 U/L (P < 0.001), GGT of 138 U/L (P < 0.001), ALT of 11.9 U/L (P < 0.001), AST of 5.7 U/L (P < 0.05), and IgM of 0.70 g/L (P < 0.001) over 12 months; TB remained stable (P = 0.98). Forty‐four patients met POISE‐inclusion criteria, 39% (n = 17) of whom had 12‐month biochemical measurements. In this subset, 18% (n = 3/17) met the 12‐month POISE primary endpoint, but considering follow‐up to 19 months, 43% achieved this target (n = 9/21). Pruritus was the most commonly reported complaint. Conclusion: Use of OCA was associated with improvement in biochemical surrogates of outcome in PBC in a real‐world setting.

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“…After careful examination of the full texts, five studies were excluded because of duplicate reports for the same subjects, reports unrelated to prevalence of PBC, and no data on prevalence or incidence for PBC. Twenty-four studies were thus included in this systematic review [5][6][7][9][10][11][12][13][14][15][16][17][18][19][20][21][22][23][24][25][26][27]30] , which comprises 22 studies based on cases and 2 based on general population (Figure 1). Among 327 studies published in Chinese (searched in Chinese database), 4 based on general population were selected and no papers based on cases were found [28,29,31,32] .…”

Section: Literature Reviewmentioning

confidence: 99%

“…USA 2010-2013 6107 -adults --Kim et al [18] , [19] , 897/2073 -Lleo et al [19] , [20] , 2017 Gatselis et al [6] , Cheung et al [21] , Non-whit Wahlin et al [7] , [23] , [24] , 2018 Yoshida et al [25] , [26] , Myers et al [9] , Chong et al [10] , Baldursdottir et al [11] , 2012…”

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confidence: 99%

Epidemiology of primary biliary cholangitis and its factors: An updated systematic review

Wu¹,

Jin²,

Wang³

et al. 2019

Preprint

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Background To understand the latest data on the prevalence and incidence rates for primary biliary cholangitis (PBC) and its factors, we systematically reviewed recent studies on epidemiology of PBC.Methods Case-finding and general population-based studies reporting prevalence and/or incidence rates for PBC were systematically identified by searching electronic databases combined with manual searching.Results A total of 37,452 PBC patients from 22 studies based on cases and 6 based on general population were included, which covered over 112.62 million inhabitants and 107,490 individuals undergoing health check-ups. As for case-finding studies, global prevalence and incidence rates for PBC ranged from 2.6 to 58.2 per 100,000 inhabitants and 0.8 to 5.3 per 100,000 inhabitants/year during the past decade, in which the variable data are associated with gender, age, race and region. Of the individuals undergoing health check-ups, 1,034 were positive for type M2 antimitochondrial antibody (AMA-M2) and 150 were diagnosed as PBC, and prevalence rates of positive AMA-M2 and PBC ranged from 430.7 to 1,456.9 and 49.2 to 276.6 per 100,000 individuals. The prevalence ratios of male/female were 1:2.2 and 1:4.1 in individuals with positive AMA-M2 and in PBC patients.Conclusions Global prevalence and incidence rates for PBC vary widely, which were significantly affected by gender, age, race and region. Studies based on general population showed a much higher prevalence of PBC and AMA-M2, and the association of gender with AMA-M2 may be far less than that with PBC in real world.

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

Cited by 13 publications

References 45 publications

Increase trend in the prevalence and male‐to‐female ratio of primary biliary cholangitis, autoimmune hepatitis, and primary sclerosing cholangitis in Japan

Increase trend in the prevalence and male‐to‐female ratio of primary biliary cholangitis, autoimmune hepatitis, and primary sclerosing cholangitis in Japan

Real‐World Effectiveness of Obeticholic Acid in Patients with Primary Biliary Cholangitis

Epidemiology of primary biliary cholangitis and its factors: An updated systematic review

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