{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,5,13]],"date-time":"2026-05-13T07:27:08Z","timestamp":1778657228959,"version":"3.51.4"},"reference-count":30,"publisher":"Springer Science and Business Media LLC","issue":"1","license":[{"start":{"date-parts":[[2018,8,22]],"date-time":"2018-08-22T00:00:00Z","timestamp":1534896000000},"content-version":"tdm","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"},{"start":{"date-parts":[[2018,8,22]],"date-time":"2018-08-22T00:00:00Z","timestamp":1534896000000},"content-version":"vor","delay-in-days":0,"URL":"https:\/\/creativecommons.org\/licenses\/by\/4.0"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Sci Rep"],"abstract":"<jats:title>Abstract<\/jats:title><jats:p>Although patients with primary biliary cholangitis (PBC) experience a variety of symptoms that could impair health-related quality of life (HRQOL), no studies regarding symptoms and impact of PBC on HRQOL have been performed in Asian countries. Herein, we aimed to evaluate symptoms and HRQOL in Japanese PBC patients. We performed a multicenter, observational, cross-sectional study. The PBC-40 and the short form (SF)-36 were used as measures of symptoms and HRQOL. Four-hundred-ninety-six patients with PBC were enrolled. In the PBC-40, the average score was highest in the emotional domain, followed by the fatigue domain. The HRQOL measured using SF-36 was also impaired, especially in the physical and role-social components. After adjustments of variables, female sex, younger age at diagnosis, and lower serum albumin level were independently associated with fatigue scores, while a longer follow-up period and lower serum albumin levels were associated with itch scores.<\/jats:p>","DOI":"10.1038\/s41598-018-31063-8","type":"journal-article","created":{"date-parts":[[2018,8,16]],"date-time":"2018-08-16T09:52:32Z","timestamp":1534413152000},"update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":22,"title":["Symptoms and health-related quality of life in Japanese patients with primary biliary cholangitis"],"prefix":"10.1038","volume":"8","author":[{"given":"Minami","family":"Yagi","sequence":"first","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-6358-5283","authenticated-orcid":false,"given":"Atsushi","family":"Tanaka","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Masanori","family":"Abe","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tadashi","family":"Namisaki","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hitoshi","family":"Yoshiji","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0568-8361","authenticated-orcid":false,"given":"Atsushi","family":"Takahashi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hiromasa","family":"Ohira","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Atsumasa","family":"Komori","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Satoshi","family":"Yamagiwa","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Kentaro","family":"Kikuchi","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Tetsuya","family":"Yasunaka","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Akinobu","family":"Takaki","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yoshiyuki","family":"Ueno","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"ORCID":"https:\/\/orcid.org\/0000-0003-0902-8272","authenticated-orcid":false,"given":"Akira","family":"Honda","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Yasushi","family":"Matsuzaki","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]},{"given":"Hajime","family":"Takikawa","sequence":"additional","affiliation":[],"role":[{"role":"author","vocabulary":"crossref"}]}],"member":"297","published-online":{"date-parts":[[2018,8,22]]},"reference":[{"key":"31063_CR1","doi-asserted-by":"publisher","first-page":"1261","DOI":"10.1056\/NEJMra043898","volume":"353","author":"MM Kaplan","year":"2005","unstructured":"Kaplan, M. M. & Gershwin, M. E. Primary biliary cirrhosis. New Engl J Med 353, 1261\u20131273 (2005).","journal-title":"New Engl J Med"},{"key":"31063_CR2","doi-asserted-by":"publisher","first-page":"291","DOI":"10.1002\/hep.22906","volume":"50","author":"KD Lindor","year":"2009","unstructured":"Lindor, K. D. et al. Primary biliary cirrhosis. Hepatology 50, 291\u2013308 (2009).","journal-title":"Hepatology"},{"key":"31063_CR3","doi-asserted-by":"crossref","unstructured":"Floreani, A., Tanaka, A., Bowlus, C., Gershwin, M. E. Geoepidemiology and changing mortality in primary biliary cholangitis. 52, 655\u2013662 (2017).","DOI":"10.1007\/s00535-017-1333-2"},{"key":"31063_CR4","doi-asserted-by":"publisher","first-page":"630","DOI":"10.1007\/s00535-009-0051-9","volume":"44","author":"N Azemoto","year":"2009","unstructured":"Azemoto, N. et al. Early biochemical response to ursodeoxycholic acid predicts symptom development in patients with asymptomatic primary biliary cirrhosis. J Gastroenterol 44, 630\u2013634 (2009).","journal-title":"J Gastroenterol"},{"key":"31063_CR5","doi-asserted-by":"publisher","first-page":"250","DOI":"10.1016\/S1386-6346(01)00148-6","volume":"22","author":"T Nakano","year":"2002","unstructured":"Nakano, T. et al. Long-term prognosis of primary biliary cirrhosis (PBC) in Japan and analysis of the factors of stage progression in asymptomatic PBC (a-PBC). Hepatol Res 22, 250\u2013260 (2002).","journal-title":"Hepatol Res"},{"key":"31063_CR6","doi-asserted-by":"publisher","first-page":"399","DOI":"10.1002\/hep.26909","volume":"60","author":"U Beuers","year":"2014","unstructured":"Beuers, U., Kremer, A. E., Bolier, R. & Elferink, R. P. Pruritus in cholestasis: facts and fiction. Hepatology 60, 399\u2013407 (2014).","journal-title":"Hepatology"},{"issue":"Suppl 2","key":"31063_CR7","doi-asserted-by":"publisher","first-page":"109","DOI":"10.1159\/000440757","volume":"33","author":"L Jopson","year":"2015","unstructured":"Jopson, L. & Jones, D. E. Fatigue in Primary Biliary Cirrhosis: Prevalence, Pathogenesis and Management. Dig Dis 33(Suppl 2), 109\u2013114 (2015).","journal-title":"Dig Dis"},{"key":"31063_CR8","doi-asserted-by":"publisher","first-page":"795","DOI":"10.1016\/j.autrev.2016.03.019","volume":"15","author":"Y Sun","year":"2016","unstructured":"Sun, Y. et al. Autotaxin, Pruritus and Primary Biliary Cholangitis (PBC). Autoimmunity Rev 15, 795\u2013800 (2016).","journal-title":"Autoimmunity Rev"},{"key":"31063_CR9","doi-asserted-by":"publisher","first-page":"1622","DOI":"10.1136\/gut.2005.065862","volume":"54","author":"A Jacoby","year":"2005","unstructured":"Jacoby, A. et al. Development, validation, and evaluation of the PBC-40, a disease specific health related quality of life measure for primary biliary cirrhosis. Gut 54, 1622\u20131629 (2005).","journal-title":"Gut"},{"key":"31063_CR10","doi-asserted-by":"publisher","first-page":"560","DOI":"10.1053\/j.gastro.2012.12.005","volume":"144","author":"M Carbone","year":"2013","unstructured":"Carbone, M. et al. Sex and age are determinants of the clinical phenotype of primary biliary cirrhosis and response to ursodeoxycholic acid. Gastroenterology 144, 560\u2013569 (2013).","journal-title":"Gastroenterology"},{"key":"31063_CR11","doi-asserted-by":"publisher","first-page":"1039","DOI":"10.1111\/apt.13794","volume":"44","author":"JK Dyson","year":"2016","unstructured":"Dyson, J. K. et al. The inter-relationship of symptom severity and quality of life in 2055 patients with primary biliary cholangitis. Aliment Pharmacol Ther 44, 1039\u20131050 (2016).","journal-title":"Aliment Pharmacol Ther"},{"key":"31063_CR12","doi-asserted-by":"publisher","first-page":"273","DOI":"10.1002\/hep.26365","volume":"58","author":"GF Mells","year":"2013","unstructured":"Mells, G. F. et al. Impact of primary biliary cirrhosis on perceived quality of life: The UK-PBC national study. Hepatology 58, 273\u2013283 (2013).","journal-title":"Hepatology"},{"key":"31063_CR13","doi-asserted-by":"publisher","first-page":"776","DOI":"10.1016\/j.jhep.2005.12.012","volume":"44","author":"JL Newton","year":"2006","unstructured":"Newton, J. L., Bhala, N., Burt, J. & Jones, D. E. Characterisation of the associations and impact of symptoms in primary biliary cirrhosis using a disease specific quality of life measure. J Hepatol 44, 776\u2013783 (2006).","journal-title":"J Hepatol"},{"key":"31063_CR14","doi-asserted-by":"publisher","first-page":"125","DOI":"10.1097\/01.meg.0000252629.96043.67","volume":"19","author":"JL Newton","year":"2007","unstructured":"Newton, J. L. et al. Autonomic dysfunction in primary biliary cirrhosis correlates with fatigue severity. Eur J Gastroenterol Hepatol 19, 125\u2013132 (2007).","journal-title":"Eur J Gastroenterol Hepatol"},{"key":"31063_CR15","doi-asserted-by":"publisher","first-page":"91","DOI":"10.1002\/hep.21230","volume":"44","author":"JL Newton","year":"2006","unstructured":"Newton, J. L., Gibson, G. J., Tomlinson, M., Wilton, K. & Jones, D. Fatigue in primary biliary cirrhosis is associated with excessive daytime somnolence. Hepatology 44, 91\u201398 (2006).","journal-title":"Hepatology"},{"key":"31063_CR16","doi-asserted-by":"publisher","first-page":"1496","DOI":"10.1002\/hep.21609","volume":"45","author":"JL Newton","year":"2007","unstructured":"Newton, J. L. et al. Population prevalence and symptom associations of autonomic dysfunction in primary biliary cirrhosis. Hepatology 45, 1496\u20131505 (2007).","journal-title":"Hepatology"},{"key":"31063_CR17","doi-asserted-by":"publisher","first-page":"228","DOI":"10.1016\/j.cgh.2007.11.021","volume":"6","author":"JL Newton","year":"2008","unstructured":"Newton, J. L., Pairman, J., Sutcliffe, K., Wilton, K. & Jones, D. E. A predictive model for fatigue and its etiologic associations in primary biliary cirrhosis. Clin Gastroenterol Hepatol 6, 228\u2013233 (2008).","journal-title":"Clin Gastroenterol Hepatol"},{"key":"31063_CR18","doi-asserted-by":"publisher","first-page":"636","DOI":"10.1111\/liv.12560","volume":"35","author":"C Quarneti","year":"2015","unstructured":"Quarneti, C. et al. Fatigue and pruritus at onset identify a more aggressive subset of primary biliary cirrhosis. Liver Int 35, 636\u2013641 (2015).","journal-title":"Liver Int"},{"key":"31063_CR19","doi-asserted-by":"publisher","first-page":"1836","DOI":"10.1002\/hep.21953","volume":"46","author":"C Selmi","year":"2007","unstructured":"Selmi, C. et al. Quality of life and everyday activities in patients with primary biliary cirrhosis. Hepatology 46, 1836\u20131843 (2007).","journal-title":"Hepatology"},{"key":"31063_CR20","doi-asserted-by":"publisher","first-page":"1114","DOI":"10.1016\/S0140-6736(17)30319-7","volume":"389","author":"VS Hegade","year":"2017","unstructured":"Hegade, V. S. et al. Effect of ileal bile acid transporter inhibitor GSK2330672 on pruritus in primary biliary cholangitis: a double-blind, randomised, placebo-controlled, crossover, phase 2a study. Lancet 389, 1114\u20131123 (2017).","journal-title":"Lancet"},{"key":"31063_CR21","doi-asserted-by":"publisher","first-page":"631","DOI":"10.1056\/NEJMoa1509840","volume":"375","author":"F Nevens","year":"2016","unstructured":"Nevens, F. et al. A Placebo-Controlled Trial of Obeticholic Acid in Primary Biliary Cholangitis. New Engl J Med 375, 631\u2013643 (2016).","journal-title":"New Engl J Med"},{"key":"31063_CR22","doi-asserted-by":"publisher","first-page":"A38","DOI":"10.1016\/j.dld.2007.12.095","volume":"40","author":"L Montali","year":"2008","unstructured":"Montali, L. et al. Validation of the Italian version of FISK Fatigue Severity Score (FFSS) and PBC-40. Digest Liver Dis 40, A38 (2008).","journal-title":"Digest Liver Dis"},{"key":"31063_CR23","doi-asserted-by":"publisher","first-page":"471","DOI":"10.1016\/j.clinre.2015.10.006","volume":"40","author":"J Raszeja-Wyszomirska","year":"2016","unstructured":"Raszeja-Wyszomirska, J. et al. Assessment of health related quality of life in polish patients with primary biliary cirrhosis. Clin Res Hepatol Gastroenterol 40, 471\u2013479 (2016).","journal-title":"Clin Res Hepatol Gastroenterol"},{"key":"31063_CR24","doi-asserted-by":"publisher","first-page":"e0175697","DOI":"10.1371\/journal.pone.0175697","volume":"12","author":"S Zec","year":"2017","unstructured":"Zec, S. et al. Translation and validation of the Serbian primary biliary cholangitis-40 questionnaire. PloS one 12, e0175697 (2017).","journal-title":"PloS one"},{"key":"31063_CR25","doi-asserted-by":"publisher","first-page":"457","DOI":"10.2957\/kanzo.57.457","volume":"57","author":"A Tanaka","year":"2016","unstructured":"Tanaka, A. et al. The assessment of subjective symptoms and patient-reported outcomes in patients with primary biliary cholangitis using PBC-40. Kanzo [in Japanese] 57, 457\u2013467 (2016).","journal-title":"Kanzo [in Japanese]"},{"key":"31063_CR26","doi-asserted-by":"publisher","first-page":"1037","DOI":"10.1016\/S0895-4356(98)00095-X","volume":"51","author":"S Fukuhara","year":"1998","unstructured":"Fukuhara, S., Bito, S., Green, J. & Hsiao, A. Kurokawa K. Translation, adaptation, and validation of the SF-36 Health Survey for use in Japan. J Clin Epidemiol 51, 1037\u20131044 (1998).","journal-title":"J Clin Epidemiol"},{"key":"31063_CR27","doi-asserted-by":"publisher","first-page":"1045","DOI":"10.1016\/S0895-4356(98)00096-1","volume":"51","author":"S Fukuhara","year":"1998","unstructured":"Fukuhara, S. et al. Psychometric and clinical tests of validity of the Japanese SF-36 Health Survey. J Clin Epidemiol 51, 1045\u20131053 (1998).","journal-title":"J Clin Epidemiol"},{"key":"31063_CR28","doi-asserted-by":"publisher","first-page":"675","DOI":"10.1007\/s00535-014-0998-z","volume":"50","author":"A Tanaka","year":"2015","unstructured":"Tanaka, A., Hirohara, J., Nakanuma, Y., Tsubouchi, H. & Takikawa, H. Biochemical responses to bezafibrate improve long-term outcome in asymptomatic patients with primary biliary cirrhosis refractory to UDCA. J Gastroenterol 50, 675\u2013682 (2015).","journal-title":"J Gastroenterol"},{"issue":"Suppl S1","key":"31063_CR29","doi-asserted-by":"publisher","first-page":"71","DOI":"10.1111\/hepr.12270","volume":"44","author":"Working Subgroup for Clinical Practice Guidelines for Primary Biliary C","year":"2014","unstructured":"Working Subgroup for Clinical Practice Guidelines for Primary Biliary C. Guidelines for the management of primary biliary cirrhosis: The Intractable Hepatobiliary Disease Study Group supported by the Ministry of Health, Labour and Welfare of Japan. Hepatol Res 44(Suppl S1), 71\u201390 (2014).","journal-title":"Hepatol Res"},{"key":"31063_CR30","unstructured":"Ware, J. E. J. The SF-36 health survey. In: Spilker B, editor. Quality of Life and Pharmacoeconomics in Clinical Trials Second edition. Philadelphia: Lippincott-Raven (1996)."}],"container-title":["Scientific Reports"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/www.nature.com\/articles\/s41598-018-31063-8.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.nature.com\/articles\/s41598-018-31063-8","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/www.nature.com\/articles\/s41598-018-31063-8.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2022,12,21]],"date-time":"2022-12-21T08:32:14Z","timestamp":1671611534000},"score":1,"resource":{"primary":{"URL":"https:\/\/www.nature.com\/articles\/s41598-018-31063-8"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2018,8,22]]},"references-count":30,"journal-issue":{"issue":"1","published-online":{"date-parts":[[2018,12]]}},"alternative-id":["31063"],"URL":"https:\/\/doi.org\/10.1038\/s41598-018-31063-8","relation":{},"ISSN":["2045-2322"],"issn-type":[{"value":"2045-2322","type":"electronic"}],"subject":[],"published":{"date-parts":[[2018,8,22]]},"assertion":[{"value":"15 May 2018","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"8 August 2018","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"22 August 2018","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"The authors declare no competing interests.","order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Competing Interests"}}],"article-number":"12542"}}