JHEP Reports (Aug 2024)

Incidence and prevalence of primary biliary cholangitis in the Netherlands – A nationwide cohort study

  • Rozanne C. de Veer,
  • Maria C.B. van Hooff,
  • Ellen Werner,
  • Ulrich Beuers,
  • Joost P.H. Drenth,
  • Frans J.C. Cuperus,
  • Bart van Hoek,
  • Bart J. Veldt,
  • Michael Klemt-Kropp,
  • Suzanne van Meer,
  • Robert C. Verdonk,
  • Hajo J. Flink,
  • Jan Maarten Vrolijk,
  • Tom J.G. Gevers,
  • Cyriel Y. Ponsioen,
  • Martijn J. ter Borg,
  • Khalida Soufidi,
  • Femke Boersma,
  • Hendrik J.M. de Jonge,
  • Frank H.J. Wolfhagen,
  • L.C. Baak,
  • Susanne L. Onderwater,
  • Jeroen D. van Bergeijk,
  • Paul G. van Putten,
  • Gijs J. de Bruin,
  • Rob P.R. Adang,
  • Maria N. Aparicio-Pages,
  • Wink de Boer,
  • Frank ter Borg,
  • Hanneke van Soest,
  • Harry L.A. Janssen,
  • Bettina E. Hansen,
  • Nicole S. Erler,
  • Adriaan J. van der Meer,
  • Sunje Abraham,
  • Rob P.R. Adang,
  • Huseyin Aktas,
  • Yasser A. Alderlieste,
  • Maria N. Aparicio-Pages,
  • L. (Bert) C. Baak,
  • Martine A.M.C. Baven-Pronk,
  • A. (Sander) van der Beek,
  • Frank C. Bekkering,
  • Jeroen D. van Bergeijk,
  • Ulrich Beuers,
  • Menno Beukema,
  • Wink de Boer,
  • Femke Boersma,
  • Kirsten Boonstra,
  • Frank ter Borg,
  • Martijn J. ter Borg,
  • Pieter C.J. ter Borg,
  • Gijs J. de Bruin,
  • Paul J. Bus,
  • Djuna L. Cahen,
  • Marcel Cazemier,
  • Frans J.C. Cuperus,
  • Lisette J.H. van Dam,
  • Maaike J. Denters,
  • Joost P.H. Drenth,
  • Ludger S.M. Epping,
  • Nicole S. Erler,
  • Hajo J. Flink,
  • Philip W. Friederich,
  • Nicole F.M. van Gerven,
  • Tom J.G. Gevers,
  • Bettina E. Hansen,
  • Sven J. van den Hazel,
  • Bart van Hoek,
  • Maria C. van Hooff,
  • Daphne M. Hotho,
  • Harry L.A. Janssen,
  • Hendrik J.M. de Jonge,
  • Matthias C. Jurgens,
  • J.(Netty) van Kemenade,
  • Marjo J. Kerbert-Dreteler,
  • Michael Klemt-Kropp,
  • Ingrid C.A.W. Konings,
  • Sander de Kort,
  • Edith M.M. Kuiper,
  • Johan P.H. Kuyvenhoven,
  • Adriaan J. van der Meer,
  • Suzanne van Meer,
  • Susanne L. Onderwater,
  • Leendert H. Oterdoom,
  • Cyriel Y. Ponsioen,
  • Paul G. van Putten,
  • Janne E. van Rooij,
  • Robert Roomer,
  • Johannes Schmidt-Böhmer,
  • Stephan Schmittgens,
  • Tim C.M.A. Schreuder,
  • Jerome Sint Nicolaas,
  • Hanneke van Soest,
  • Khalida Soufidi,
  • Stephan H.C. van Stiphout,
  • Hans H.K. Thio,
  • Merel M. Tielemans,
  • Sigrid Vandebosch,
  • Rozanne C. de Veer,
  • Bart J. Veldt,
  • Robert C. Verdonk,
  • J. Marleen de Vree,
  • Elsemieke de Vries,
  • Anne Vrieze,
  • Jan Maarten Vrolijk,
  • Laurens A. van der Waaij,
  • Ellen Werner,
  • Ulrike de Wit,
  • Frank H.J. Wolfhagen

Journal volume & issue
Vol. 6, no. 8
p. 101132

Abstract

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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.

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