Disease burden in primary sclerosing cholangitis in the Netherlands: A long-term follow-up study

Kim N. van Munster, Bregje Mol, the EpiPSC2 Study Group, Jorn C. Goet, Sanne N. van Munster, Rinse K. Weersma, Annemarie C. de Vries, Adriaan J. van der Meer, Akin Inderson, Joost P. Drenth, Karel J. van Erpecum, Kirsten Boonstra, Ulrich Beuers, Marcel G.W. Dijkgraaf, Cyriel Y. Ponsioen*

*Corresponding author for this work

Research output: Contribution to journalArticleAcademicpeer-review

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Background & Aims: Primary sclerosing cholangitis (PSC) is a progressive, cholestatic liver disease which greatly impacts the lives of individuals. Burden of disease due to shortened life expectancy and impaired quality of life is ill-described. The aim of this study was to assess long-term disease burden in a large population-based registry with regard to survival, clinical course, quality adjusted life years (QALYs), medical consumption and work productivity loss. Methods: All PSC patients living in a geographically defined area covering ~50% of the Netherlands were included, together with patients from the three liver transplant centres. Survival was estimated by competing risk analysis. Proportional shortfall of QALYs during disease course was measured relative to a matched reference cohort using validated questionnaires. Work productivity loss and medical consumption were evaluated over time. Results: A total of 1208 patients were included with a median follow-up of 11.2 year. Median liver transplant-free survival was 21.0 years. Proportional shortfall of QALYs increased to 48% >25 years after diagnosis. Patients had on average 12.4 hospital contact days among which 3.17 admission days per year, annual medical costs were €12 169 and mean work productivity loss was 25%. Conclusions: Our data quantify for the first time disease burden in terms of QALYs lost, clinical events, medical consumption, costs as well as work productivity loss, and show that all these are substantial and increase over time.

Original languageEnglish
Pages (from-to)639-648
Number of pages10
JournalLiver International
Issue number3
Early online date3 Nov 2022
Publication statusPublished - Mar 2023

Bibliographical note

Funding Information:
The study was funded by the Netherlands Organisation for Health Research and Development (ZonMw, grant number: 836041010).

Publisher Copyright:
© 2022 The Authors. Liver International published by John Wiley & Sons Ltd.


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