{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,23]],"date-time":"2026-09-23T02:36:01Z","timestamp":1790130961435,"version":"4.0.1"},"reference-count":39,"publisher":"Wiley","issue":"3","license":[{"start":{"date-parts":[[2022,11,15]],"date-time":"2022-11-15T00:00:00Z","timestamp":1668470400000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/creativecommons.org\/licenses\/by\/4.0\/"}],"funder":[{"DOI":"10.13039\/501100001826","name":"ZonMw","doi-asserted-by":"publisher","id":[{"id":"10.13039\/501100001826","id-type":"DOI","asserted-by":"publisher"}]}],"content-domain":{"domain":["onlinelibrary.wiley.com"],"crossmark-restriction":true},"short-container-title":["Liver International"],"published-print":{"date-parts":[[2023,3]]},"abstract":"<jats:title>Abstract<\/jats:title><jats:sec><jats:title>Background &amp; Aims<\/jats:title><jats:p>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\u2010described. The aim of this study was to assess long\u2010term disease burden in a large population\u2010based registry with regard to survival, clinical course, quality adjusted life years (QALYs), medical consumption and work productivity loss.<\/jats:p><\/jats:sec><jats:sec><jats:title>Methods<\/jats:title><jats:p>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.<\/jats:p><\/jats:sec><jats:sec><jats:title>Results<\/jats:title><jats:p>A total of 1208 patients were included with a median follow\u2010up of 11.2\u00a0year. Median liver transplant\u2010free survival was 21.0\u2009years. Proportional shortfall of QALYs increased to 48% &gt;25\u2009years after diagnosis. Patients had on average 12.4 hospital contact days among which 3.17 admission days per year, annual medical costs were \u20ac12\u2009169 and mean work productivity loss was 25%.<\/jats:p><\/jats:sec><jats:sec><jats:title>Conclusions<\/jats:title><jats:p>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.<\/jats:p><\/jats:sec>","DOI":"10.1111\/liv.15471","type":"journal-article","created":{"date-parts":[[2022,11,4]],"date-time":"2022-11-04T03:03:30Z","timestamp":1667531010000},"page":"639-648","update-policy":"https:\/\/doi.org\/10.1002\/crossmark_policy","source":"Crossref","is-referenced-by-count":20,"title":["Disease burden in primary sclerosing cholangitis in the Netherlands: A long\u2010term follow\u2010up study"],"prefix":"10.1111","volume":"43","author":[{"ORCID":"https:\/\/orcid.org\/0000-0001-7019-5083","authenticated-orcid":false,"given":"Kim N.","family":"van Munster","sequence":"first","affiliation":[{"name":"Department of Gastroenterology and Hepatology Amsterdam University Medical Center, location AMC, Amsterdam Gastroenterology, Endocrinology &amp; Metabolism  Amsterdam The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bregje","family":"Mol","sequence":"additional","affiliation":[{"name":"Department of Gastroenterology and Hepatology Amsterdam University Medical Center, location AMC, Amsterdam Gastroenterology, Endocrinology &amp; Metabolism  Amsterdam The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Jorn C.","family":"Goet","sequence":"additional","affiliation":[{"name":"Department of Gastroenterology and Hepatology, Erasmus MC University Medical Center  Rotterdam The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Sanne N.","family":"van Munster","sequence":"additional","affiliation":[{"name":"Department of Gastroenterology and Hepatology Amsterdam University Medical Center, location AMC, Amsterdam Gastroenterology, Endocrinology &amp; Metabolism  Amsterdam The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Rinse K.","family":"Weersma","sequence":"additional","affiliation":[{"name":"Department of Gastroenterology and Hepatology University of Groningen and University Medical Center Groningen  Groningen The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Annemarie C.","family":"de Vries","sequence":"additional","affiliation":[{"name":"Department of Gastroenterology and Hepatology, Erasmus MC University Medical Center  Rotterdam The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Adriaan J.","family":"van der Meer","sequence":"additional","affiliation":[{"name":"Department of Gastroenterology and Hepatology, Erasmus MC University Medical Center  Rotterdam The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Akin","family":"Inderson","sequence":"additional","affiliation":[{"name":"Department of Gastroenterology and Hepatology Leiden University Medical Center  Leiden The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-8027-3073","authenticated-orcid":false,"given":"Joost P.","family":"Drenth","sequence":"additional","affiliation":[{"name":"Department of Gastroenterology and Hepatology Radboud University Medical Center  Nijmegen The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Karel J.","family":"van Erpecum","sequence":"additional","affiliation":[{"name":"Department of Gastroenterology and Hepatology University Medical Center Utrecht  Utrecht The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Kirsten","family":"Boonstra","sequence":"additional","affiliation":[{"name":"Department of Gastroenterology and Hepatology Amsterdam University Medical Center, location VUmc, Amsterdam Gastroenterology &amp; Metabolism  Amsterdam The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5114-7799","authenticated-orcid":false,"given":"Ulrich","family":"Beuers","sequence":"additional","affiliation":[{"name":"Department of Gastroenterology and Hepatology Amsterdam University Medical Center, location AMC, Amsterdam Gastroenterology, Endocrinology &amp; Metabolism  Amsterdam The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marcel G. W.","family":"Dijkgraaf","sequence":"additional","affiliation":[{"name":"Department of Epidemiology and Data Science Amsterdam University Medical Center, location University of Amsterdam, Amsterdam Public Health  Amsterdam The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0001-5203-0453","authenticated-orcid":false,"given":"Cyriel Y.","family":"Ponsioen","sequence":"additional","affiliation":[{"name":"Department of Gastroenterology and Hepatology Amsterdam University Medical Center, location AMC, Amsterdam Gastroenterology, Endocrinology &amp; Metabolism  Amsterdam The Netherlands"}],"role":[{"vocabulary":"crossref","role":"author"}]},{"name":"the EpiPSC2 Study Group","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"311","published-online":{"date-parts":[[2022,11,15]]},"reference":[{"key":"e_1_2_10_2_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0140-6736(18)30300-3"},{"key":"e_1_2_10_3_1","doi-asserted-by":"publisher","DOI":"10.1136\/gut.38.4.610"},{"key":"e_1_2_10_4_1","doi-asserted-by":"publisher","DOI":"10.1111\/apt.15148"},{"key":"e_1_2_10_5_1","doi-asserted-by":"publisher","DOI":"10.1002\/hep.26565"},{"key":"e_1_2_10_6_1","doi-asserted-by":"publisher","DOI":"10.1053\/j.gastro.2017.02.038"},{"key":"e_1_2_10_7_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jhep.2009.04.009"},{"key":"e_1_2_10_8_1","doi-asserted-by":"publisher","DOI":"10.1002\/hep.22322"},{"key":"e_1_2_10_9_1","doi-asserted-by":"publisher","DOI":"10.3109\/00365528909091339"},{"key":"e_1_2_10_10_1","doi-asserted-by":"publisher","DOI":"10.1111\/liv.13110"},{"key":"e_1_2_10_11_1","volume-title":"Confidence Intervals for a Rate","author":"Sullivan KMSM","year":"2006"},{"key":"e_1_2_10_12_1","doi-asserted-by":"publisher","DOI":"10.1186\/1742-5573-1-6"},{"key":"e_1_2_10_13_1","unstructured":"KevinM.SullivanAGD.PersonTime2\u2014Comparing Two Person\u2010Time Rates.https:\/\/testopenepi.com\/PersonTime2\/PersonTime2.htm."},{"key":"e_1_2_10_14_1","unstructured":"GrayB.Subdistribution analysis of competing risks.2022.https:\/\/cran.r\u2010project.org\/web\/packages\/cmprsk\/cmprsk.pdf."},{"key":"e_1_2_10_15_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jval.2016.01.003"},{"key":"e_1_2_10_16_1","unstructured":"CBS.Statline.https:\/\/opendata.cbs.nl\/statline\/#\/CBS\/nl\/."},{"key":"e_1_2_10_17_1","unstructured":"BouwmansC KoopmanschapMA KrolM et al.Handleiding iMTA Medical Cost Questionnaire (iMCQ).2013."},{"key":"e_1_2_10_18_1","volume-title":"Cost Guide. Cost Research Methodology and Reference Prices for Economic Evaluations in Health Care","author":"Hakkaart\u2010van Roijen LVLN","year":"2015"},{"key":"e_1_2_10_19_1","unstructured":"STATLINE C.Consumer prizes; price index 2015=100.https:\/\/opendata.cbs.nl\/statline\/?dl=3F0E#\/CBS\/nl\/dataset\/83131NED\/table"},{"key":"e_1_2_10_20_1","unstructured":"RIVM.[Cost of illness]. 29 oktober 2019 2019.https:\/\/statline.rivm.nl\/#\/RIVM\/nl\/dataset\/50050NED\/table?ts=1608275685025"},{"key":"e_1_2_10_21_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jval.2015.05.009"},{"key":"e_1_2_10_22_1","unstructured":"IBM.SPSS Statistics for Windows Version 26.0;2017."},{"key":"e_1_2_10_23_1","volume-title":"RStudio: integrated development for R","author":"Team R","year":"2020"},{"key":"e_1_2_10_24_1","doi-asserted-by":"publisher","DOI":"10.1080\/00365521.2019.1707277"},{"key":"e_1_2_10_25_1","doi-asserted-by":"publisher","DOI":"10.1111\/j.1572-0241.2001.03537.x"},{"key":"e_1_2_10_26_1","doi-asserted-by":"publisher","DOI":"10.1177\/0272989X08315240"},{"key":"e_1_2_10_27_1","unstructured":"OECD.Purchasing power parities (PPP) (indicator).2020.https:\/\/www.oecd\u2010ilibrary.org\/finance\u2010and\u2010investment\/purchasing\u2010power\u2010parities\u2010ppp\/indicator\/english_1290ee5a\u2010en."},{"key":"e_1_2_10_28_1","unstructured":"Eurostat.Wages and labour costs. 2020.https:\/\/ec.europa.eu\/eurostat\/statistics\u2010explained\/index.php?title=Wages_and_labour_costs"},{"key":"e_1_2_10_29_1","doi-asserted-by":"publisher","DOI":"10.1016\/S2468-1253(20)30012-1"},{"key":"e_1_2_10_30_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.dld.2019.12.126"},{"key":"e_1_2_10_31_1","doi-asserted-by":"publisher","DOI":"10.1371\/journal.pone.0182391"},{"key":"e_1_2_10_32_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.cgh.2016.07.020"},{"key":"e_1_2_10_33_1","unstructured":"CBS.Employee sickness absence increased further in 2019. 16\u201003\u20102020 2020.https:\/\/www.cbs.nl\/en\u2010gb\/news\/2020\/12\/employee\u2010sickness\u2010absence\u2010increased\u2010further\u2010in\u20102019"},{"key":"e_1_2_10_34_1","doi-asserted-by":"publisher","DOI":"10.1016\/j.jhep.2012.10.013"},{"key":"e_1_2_10_35_1","doi-asserted-by":"publisher","DOI":"10.1111\/liv.14644"},{"key":"e_1_2_10_36_1","doi-asserted-by":"publisher","DOI":"10.1111\/liv.15271"},{"key":"e_1_2_10_37_1","doi-asserted-by":"publisher","DOI":"10.1186\/s41687-022-00484-5"},{"key":"e_1_2_10_38_1","unstructured":"VijgenSVHF ObradovicM.Disease burden in daily practice. The theory and practice of calculation of disease burden during assessments of the health care package. Diemen;2018."},{"key":"e_1_2_10_39_1","doi-asserted-by":"publisher","DOI":"10.1017\/S0266462305050397"},{"key":"e_1_2_10_40_1","doi-asserted-by":"publisher","DOI":"10.1016\/S0895-4356(99)00177-8"}],"container-title":["Liver International"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/liv.15471","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/full-xml\/10.1111\/liv.15471","content-type":"application\/xml","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/pdf\/10.1111\/liv.15471","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2023,8,18]],"date-time":"2023-08-18T16:55:39Z","timestamp":1692377739000},"score":1,"resource":{"primary":{"URL":"https:\/\/onlinelibrary.wiley.com\/doi\/10.1111\/liv.15471"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2022,11,15]]},"references-count":39,"journal-issue":{"issue":"3","published-print":{"date-parts":[[2023,3]]}},"alternative-id":["10.1111\/liv.15471"],"URL":"https:\/\/doi.org\/10.1111\/liv.15471","archive":["Portico"],"relation":{},"ISSN":["1478-3223","1478-3231"],"issn-type":[{"value":"1478-3223","type":"print"},{"value":"1478-3231","type":"electronic"}],"subject":[],"published":{"date-parts":[[2022,11,15]]},"assertion":[{"value":"2022-07-06","order":0,"name":"received","label":"Received","group":{"name":"publication_history","label":"Publication History"}},{"value":"2022-10-19","order":1,"name":"accepted","label":"Accepted","group":{"name":"publication_history","label":"Publication History"}},{"value":"2022-11-15","order":2,"name":"published","label":"Published","group":{"name":"publication_history","label":"Publication History"}}]}}