TY - JOUR
T1 - Incidence and prevalence of primary biliary cholangitis in the Netherlands – A nationwide cohort study
AU - de Veer, Rozanne C.
AU - van Hooff, Maria C.
AU - the Dutch PBC Study Group
AU - Werner, Ellen
AU - Beuers, Ulrich
AU - Drenth, Joost P.H.
AU - Cuperus, Frans J.C.
AU - van Hoek, Bart
AU - Veldt, Bart J.
AU - Klemt-Kropp, Michael
AU - van Meer, Suzanne
AU - Verdonk, Robert C.
AU - Flink, Hajo J.
AU - Vrolijk, Jan Maarten
AU - Gevers, Tom J.G.
AU - Ponsioen, Cyriel Y.
AU - ter Borg, Martijn J.
AU - Soufidi, Khalida
AU - Boersma, Femke
AU - de Jonge, Hendrik J.M.
AU - Wolfhagen, Frank H.J.
AU - Baak, L. C.
AU - Onderwater, Susanne L.
AU - van Bergeijk, Jeroen D.
AU - van Putten, Paul G.
AU - de Bruin, Gijs J.
AU - Adang, Rob P.R.
AU - Aparicio-Pages, Maria N.
AU - de Boer, Wink
AU - ter Borg, Frank
AU - van Soest, Hanneke
AU - Janssen, Harry L.A.
AU - Hansen, Bettina E.
AU - Erler, Nicole S.
AU - van der Meer, Adriaan J.
AU - Abraham, Sunje
AU - Aktas, Huseyin
AU - van der Beek, A. (Sander)
AU - Cahen, Djuna L.
AU - Denters, Maaike J.
AU - van Gerven, Nicole F.M.
AU - van Hooff, Maria C.
AU - Hotho, Daphne M.
AU - Konings, Ingrid C.A.W.
AU - Kuiper, Edith M.M.
AU - Oterdoom, Leendert H.
AU - Roomer, Robert
AU - Nicolaas, Jerome Sint
AU - Tielemans, Merel M.
AU - de Vries, Elsemieke
AU - Vrieze, Anne
N1 - Publisher Copyright:
© 2024 The Authors
PY - 2024/8
Y1 - 2024/8
N2 - Background & Aims: Although primary biliary cholangitis (PBC) is considered a rare disorder, accurate determination of its incidence and prevalence remains challenging due to limited comprehensive population-based registries. We aimed to assess the incidence and prevalence of PBC in the Netherlands over time through the nationwide Dutch PBC Cohort Study (DPCS). Methods: DPCS retrospectively included every identifiable patient with PBC in the Netherlands from 1990 onwards in all 71 Dutch hospitals. Incidence and prevalence were assessed between 2008-2018 by Poisson regression between sex and age groups over time. Results: On the 1st of January 2008, there were 1,458 patients with PBC in the Netherlands. Between 2008-2018, 2,187 individuals were newly diagnosed, 46 were transplanted and 468 died. The yearly incidence of PBC in 2008 was 1.38, increasing to 1.74 per 100,000 persons in 2018. When compared to those aged <45 years, females aged 45-64 years (adjusted incidence rate ratio 4.21, 95% CI 3.76-4.71, p <0.001) and males ≥65 years (adjusted incidence rate ratio 14.41, 95% CI 9.62-21.60, p <0.001) were at the highest risk of being diagnosed with PBC. The male-to-female ratio of patients newly diagnosed with PBC during the study period was 1:14 in those <45 years, 1:10 in patients aged 45-64 years, and 1:4 in those ≥65 years. Point prevalence increased from 11.9 in 2008 to 21.5 per 100,000 persons in 2018. Average annual percent change in this time period was 5.94% (95% CI 5.77-6.15, p <0.05), and was the highest among the population aged ≥65 years (5.69%, 95% CI 5.32-6.36, p <0.001). Conclusions: In this nationwide cohort study, we observed an increase in both the incidence and prevalence of PBC in the Netherlands over the past decade, with marked age and sex differences. Impact and implications: This nationwide Dutch primary biliary cholangitis (PBC) Cohort Study, including all hospitals in the Netherlands, showed that the incidence and prevalence of PBC have increased over the last decade. The age-dependent PBC incidence rate differed for males (highest risk ≥65 years) and females (highest risk between 45 and 65 years), which may be related to a difference in the timing of exposure to environmental triggers of PBC. The largest increase in PBC prevalence over time was observed in the population aged ≥65 years, which may have implications for the use of second-line therapies. These results therefore indicate that further studies are needed to elaborate on the advantages and disadvantages of add-on therapies in the elderly population.
AB - Background & Aims: Although primary biliary cholangitis (PBC) is considered a rare disorder, accurate determination of its incidence and prevalence remains challenging due to limited comprehensive population-based registries. We aimed to assess the incidence and prevalence of PBC in the Netherlands over time through the nationwide Dutch PBC Cohort Study (DPCS). Methods: DPCS retrospectively included every identifiable patient with PBC in the Netherlands from 1990 onwards in all 71 Dutch hospitals. Incidence and prevalence were assessed between 2008-2018 by Poisson regression between sex and age groups over time. Results: On the 1st of January 2008, there were 1,458 patients with PBC in the Netherlands. Between 2008-2018, 2,187 individuals were newly diagnosed, 46 were transplanted and 468 died. The yearly incidence of PBC in 2008 was 1.38, increasing to 1.74 per 100,000 persons in 2018. When compared to those aged <45 years, females aged 45-64 years (adjusted incidence rate ratio 4.21, 95% CI 3.76-4.71, p <0.001) and males ≥65 years (adjusted incidence rate ratio 14.41, 95% CI 9.62-21.60, p <0.001) were at the highest risk of being diagnosed with PBC. The male-to-female ratio of patients newly diagnosed with PBC during the study period was 1:14 in those <45 years, 1:10 in patients aged 45-64 years, and 1:4 in those ≥65 years. Point prevalence increased from 11.9 in 2008 to 21.5 per 100,000 persons in 2018. Average annual percent change in this time period was 5.94% (95% CI 5.77-6.15, p <0.05), and was the highest among the population aged ≥65 years (5.69%, 95% CI 5.32-6.36, p <0.001). Conclusions: In this nationwide cohort study, we observed an increase in both the incidence and prevalence of PBC in the Netherlands over the past decade, with marked age and sex differences. Impact and implications: This nationwide Dutch primary biliary cholangitis (PBC) Cohort Study, including all hospitals in the Netherlands, showed that the incidence and prevalence of PBC have increased over the last decade. The age-dependent PBC incidence rate differed for males (highest risk ≥65 years) and females (highest risk between 45 and 65 years), which may be related to a difference in the timing of exposure to environmental triggers of PBC. The largest increase in PBC prevalence over time was observed in the population aged ≥65 years, which may have implications for the use of second-line therapies. These results therefore indicate that further studies are needed to elaborate on the advantages and disadvantages of add-on therapies in the elderly population.
UR - http://www.scopus.com/inward/record.url?scp=85198550936&partnerID=8YFLogxK
U2 - 10.1016/j.jhepr.2024.101132
DO - 10.1016/j.jhepr.2024.101132
M3 - Article
C2 - 39113899
AN - SCOPUS:85198550936
SN - 2589-5559
VL - 6
JO - JHEP Reports
JF - JHEP Reports
IS - 8
M1 - 101132
ER -