{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,6]],"date-time":"2026-09-06T05:02:15Z","timestamp":1788670935475,"version":"build-2803163510"},"reference-count":26,"publisher":"Springer Science and Business Media LLC","issue":"10","license":[{"start":{"date-parts":[[2019,12,18]],"date-time":"2019-12-18T00:00:00Z","timestamp":1576627200000},"content-version":"tdm","delay-in-days":0,"URL":"http:\/\/www.springer.com\/tdm"},{"start":{"date-parts":[[2019,12,18]],"date-time":"2019-12-18T00:00:00Z","timestamp":1576627200000},"content-version":"vor","delay-in-days":0,"URL":"http:\/\/www.springer.com\/tdm"}],"content-domain":{"domain":["link.springer.com"],"crossmark-restriction":false},"short-container-title":["Dig Dis Sci"],"published-print":{"date-parts":[[2020,10]]},"DOI":"10.1007\/s10620-019-06009-3","type":"journal-article","created":{"date-parts":[[2019,12,18]],"date-time":"2019-12-18T14:02:48Z","timestamp":1576677768000},"page":"3006-3013","update-policy":"https:\/\/doi.org\/10.1007\/springer_crossmark_policy","source":"Crossref","is-referenced-by-count":26,"title":["Symptom Burden and Treatment Response in Patients with Primary Biliary Cholangitis (PBC)"],"prefix":"10.1007","volume":"65","author":[{"given":"Leonard","family":"Kaps","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Annette","family":"Grambihler","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Bethlehem","family":"Yemane","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael","family":"Nagel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Christian","family":"Labenz","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Pascal","family":"Ploch","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Maurice","family":"Michel","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Peter R.","family":"Galle","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Marcus-Alexander","family":"W\u00f6rns","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"ORCID":"https:\/\/orcid.org\/0000-0002-4224-4703","authenticated-orcid":false,"given":"J\u00f6rn M.","family":"Schattenberg","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"297","published-online":{"date-parts":[[2019,12,18]]},"reference":[{"key":"6009_CR1","doi-asserted-by":"publisher","first-page":"303","DOI":"10.1146\/annurev-pathol-020712-164014","volume":"8","author":"GM Hirschfield","year":"2013","unstructured":"Hirschfield GM, Gershwin ME. The immunobiology and pathophysiology of primary biliary cirrhosis. Annu Rev Pathol Mech. 2013;8:303\u2013330.","journal-title":"Annu Rev Pathol Mech"},{"key":"6009_CR2","doi-asserted-by":"publisher","first-page":"45","DOI":"10.1007\/s11894-002-0037-8","volume":"4","author":"A Mason","year":"2002","unstructured":"Mason A, Nair S. Primary biliary cirrhosis: new thoughts on pathophysiology and treatment. Curr Gastroenterol Rep. 2002;4:45\u201351.","journal-title":"Curr Gastroenterol Rep"},{"key":"6009_CR3","doi-asserted-by":"publisher","first-page":"273","DOI":"10.1002\/hep.26365","volume":"58","author":"GF Mells","year":"2013","unstructured":"Mells GF, Pells G, Newton JL, et al. Impact of primary biliary cirrhosis on perceived quality of life: the UK-PBC national study. Hepatology. 2013;58:273\u2013283.","journal-title":"Hepatology"},{"key":"6009_CR4","doi-asserted-by":"publisher","first-page":"2076","DOI":"10.1016\/j.cgh.2018.12.028","volume":"10","author":"AC Cheung","year":"2019","unstructured":"Cheung AC, Lammers WJ, Murillo Perez CF, et al. Effects of age and sex of response to ursodeoxycholic acid and transplant-free survival in patients with primary biliary cholangitis. Clin Gastroenterol Hepatol. 2019;10:2076\u20132084.","journal-title":"Clin Gastroenterol Hepatol"},{"key":"6009_CR5","doi-asserted-by":"publisher","first-page":"357","DOI":"10.1016\/j.jhep.2019.04.001","volume":"71","author":"MH Harms","year":"2019","unstructured":"Harms MH, van Buuren HR, Corpechot C, et al. Ursodeoxycholic acid therapy and liver transplant-free survival in patients with primary biliary cholangitis. J Hepatol. 2019;71:357\u2013365.","journal-title":"J Hepatol"},{"key":"6009_CR6","doi-asserted-by":"publisher","first-page":"2044","DOI":"10.1111\/j.1572-0241.2006.00699.x","volume":"101","author":"PCJ ter Borg","year":"2006","unstructured":"ter Borg PCJ, Schalm SW, Hansen BE, van Buuren HR, Dutch PBC Study Group. Prognosis of ursodeoxycholic acid-treated patients with primary biliary cirrhosis. Results of a 10-yr cohort study involving 297 patients. Am J Gastroenterol. 2006;101:2044\u20132050.","journal-title":"Am J Gastroenterol"},{"key":"6009_CR7","doi-asserted-by":"publisher","first-page":"715","DOI":"10.1053\/j.gastro.2005.12.029","volume":"130","author":"A Par\u00e9s","year":"2006","unstructured":"Par\u00e9s A, Caballer\u00eda L, Rod\u00e9s J. Excellent long-term survival in patients with primary biliary cirrhosis and biochemical response to ursodeoxycholic acid. Gastroenterology. 2006;130:715\u2013720.","journal-title":"Gastroenterology"},{"key":"6009_CR8","doi-asserted-by":"publisher","first-page":"145","DOI":"10.1016\/j.jhep.2017.03.022","volume":"67","author":"GM Hirschfield","year":"2017","unstructured":"Hirschfield GM, Beuers U, Corpechot C, et al. EASL clinical practice guidelines: the diagnosis and management of patients with primary biliary cholangitis. J Hepatol. 2017;67:145\u2013172.","journal-title":"J Hepatol"},{"key":"6009_CR9","doi-asserted-by":"publisher","first-page":"609","DOI":"10.1080\/00365521.2019.1606931","volume":"54","author":"KT \u00d6rnolfsson","year":"2019","unstructured":"\u00d6rnolfsson KT, Lund SH, Olafsson S, Bergmann OM, Bj\u00f6rnsson ES. Biochemical response to ursodeoxycholic acid among PBC patients: a nationwide population-based study. Scand J Gastroenterol. 2019;54:609\u2013616.","journal-title":"Scand J Gastroenterol"},{"key":"6009_CR10","doi-asserted-by":"publisher","first-page":"1354","DOI":"10.1111\/apt.12522","volume":"38","author":"V Papastergiou","year":"2013","unstructured":"Papastergiou V, Tsochatzis EA, Rodriguez-Peralvarez M, et al. Biochemical criteria at 1 year are not robust indicators of response to ursodeoxycholic acid in early primary biliary cirrhosis: results from a 29-year cohort study. Aliment Pharmacol Ther. 2013;38:1354\u20131364.","journal-title":"Aliment Pharmacol Ther"},{"key":"6009_CR11","doi-asserted-by":"publisher","first-page":"1804","DOI":"10.1053\/j.gastro.2015.07.061","volume":"149","author":"WJ Lammers","year":"2015","unstructured":"Lammers WJ, Hirschfield GM, Corpechot C, et al. Development and validation of a scoring system to predict outcomes of patients with primary biliary cirrhosis receiving ursodeoxycholic acid therapy. Gastroenterology. 2015;149:1804\u20131812.","journal-title":"Gastroenterology"},{"key":"6009_CR12","doi-asserted-by":"publisher","first-page":"930","DOI":"10.1002\/hep.28017","volume":"63","author":"M Carbone","year":"2016","unstructured":"Carbone M, Sharp SJ, Flack S, et al. The UK-PBC risk scores: derivation and validation of a scoring system for long-term prediction of end-stage liver disease in primary biliary cholangitis. Hepatology. 2016;63:930\u2013950.","journal-title":"Hepatology"},{"key":"6009_CR13","doi-asserted-by":"publisher","first-page":"1161","DOI":"10.1080\/14656566.2019.1601701","volume":"20","author":"AA Gossard","year":"2019","unstructured":"Gossard AA, Lindor KD. Current and promising therapy for primary biliary cholangitis. Expert Opin Pharmacother. 2019;20:1161\u20131167.","journal-title":"Expert Opin Pharmacother"},{"key":"6009_CR14","doi-asserted-by":"publisher","first-page":"2171","DOI":"10.1056\/NEJMoa1714519","volume":"378","author":"C Corpechot","year":"2018","unstructured":"Corpechot C, Chazouill\u00e8res O, Rousseau A, et al. A placebo-controlled trial of bezafibrate in primary biliary cholangitis. N Engl J Med. 2018;378:2171\u20132181.","journal-title":"N Engl J Med"},{"key":"6009_CR15","doi-asserted-by":"publisher","first-page":"631","DOI":"10.1056\/NEJMoa1509840","volume":"375","author":"F Nevens","year":"2016","unstructured":"Nevens F, Andreone P, Mazzella G, et al. A placebo-controlled trial of obeticholic acid in primary biliary cholangitis. N Engl J Med. 2016;375:631\u2013643.","journal-title":"N Engl J Med"},{"key":"6009_CR16","first-page":"145","volume":"15","author":"FQ Onofrio","year":"2019","unstructured":"Onofrio FQ, Hirschfield GM, Gulamhusein AF. A practical review of primary biliary cholangitis for the gastroenterologist. Gastroenterol Hepatol N Y. 2019;15:145\u2013154.","journal-title":"Gastroenterol Hepatol N Y"},{"key":"6009_CR17","doi-asserted-by":"publisher","first-page":"1044","DOI":"10.1053\/gast.2002.36027","volume":"123","author":"M Prince","year":"2002","unstructured":"Prince M, Chetwynd A, Newman W, Metcalf JV, James OFW. Survival and symptom progression in a geographically based cohort of patients with primary biliary cirrhosis: follow-up for up to 28 years. Gastroenterology. 2002;123:1044\u20131051.","journal-title":"Gastroenterology"},{"key":"6009_CR18","doi-asserted-by":"publisher","first-page":"636","DOI":"10.1111\/liv.12560","volume":"35","author":"C Quarneti","year":"2015","unstructured":"Quarneti C, Muratori P, Lalanne C, et al. Fatigue and pruritus at onset identify a more aggressive subset of primary biliary cirrhosis. Liver Int. 2015;35:636\u2013641.","journal-title":"Liver Int"},{"key":"6009_CR19","doi-asserted-by":"publisher","first-page":"911","DOI":"10.1016\/j.jhep.2010.05.026","volume":"53","author":"DE Jones","year":"2010","unstructured":"Jones DE, Al-Rifai A, Frith J, Patanwala I, Newton JL. The independent effects of fatigue and UDCA therapy on mortality in primary biliary cirrhosis: results of a 9 year follow-up. J Hepatol. 2010;53:911\u2013917.","journal-title":"J Hepatol"},{"key":"6009_CR20","doi-asserted-by":"publisher","first-page":"716","DOI":"10.1016\/S2468-1253(17)30246-7","volume":"2","author":"D Jones","year":"2017","unstructured":"Jones D, Boudes PF, Swain MG, et al. Seladelpar (MBX-8025), a selective PPAR-\u03b4 agonist, in patients with primary biliary cholangitis with an inadequate response to ursodeoxycholic acid: a double-blind, randomised, placebo-controlled, phase 2, proof-of-concept study. Lancet Gastroenterol Hepatol. 2017;2:716\u2013726.","journal-title":"Lancet Gastroenterol Hepatol"},{"key":"6009_CR21","doi-asserted-by":"publisher","first-page":"1877","DOI":"10.1111\/liv.13477","volume":"37","author":"D Suraweera","year":"2017","unstructured":"Suraweera D, Rahal H, Jimenez M, Viramontes M, Choi G, Saab S. Treatment of primary biliary cholangitis ursodeoxycholic acid non-responders: a systematic review. Liver Int. 2017;37:1877\u20131886.","journal-title":"Liver Int"},{"key":"6009_CR22","doi-asserted-by":"publisher","first-page":"871","DOI":"10.1002\/hep.22428","volume":"48","author":"C Corpechot","year":"2008","unstructured":"Corpechot C, Abenavoli L, Rabahi N, et al. Biochemical response to ursodeoxycholic acid and long-term prognosis in primary biliary cirrhosis. Hepatology. 2008;48:871\u2013877.","journal-title":"Hepatology"},{"key":"6009_CR23","doi-asserted-by":"publisher","first-page":"776","DOI":"10.1016\/j.jhep.2005.12.012","volume":"44","author":"JL Newton","year":"2006","unstructured":"Newton JL, Bhala N, Burt J, Jones DEJ. Characterisation of the associations and impact of symptoms in primary biliary cirrhosis using a disease specific quality of life measure. J Hepatol. 2006;44:776\u2013783.","journal-title":"J Hepatol"},{"key":"6009_CR24","doi-asserted-by":"publisher","first-page":"341","DOI":"10.1016\/j.mehy.2011.11.016","volume":"78","author":"H L\u00f6vheim","year":"2012","unstructured":"L\u00f6vheim H. A new three-dimensional model for emotions and monoamine neurotransmitters. Med Hypotheses. 2012;78:341\u2013348.","journal-title":"Med Hypotheses"},{"key":"6009_CR25","doi-asserted-by":"publisher","first-page":"623","DOI":"10.1097\/00042737-199906000-00007","volume":"11","author":"EA Jones","year":"1999","unstructured":"Jones EA, Bergasa NV. The pathogenesis and treatment of pruritus and fatigue in patients with PBC. Eur J Gastroenterol Hepatol. 1999;11:623\u2013631.","journal-title":"Eur J Gastroenterol Hepatol"},{"key":"6009_CR26","doi-asserted-by":"publisher","first-page":"2085","DOI":"10.1016\/j.cgh.2018.12.016","volume":"17","author":"Y Huber","year":"2019","unstructured":"Huber Y, Boyle M, Hallsworth K, et al. Health-related quality of life in nonalcoholic fatty liver disease associates with hepatic inflammation. Clin Gastroenterol Hepatol. 2019;17:2085\u20132092.","journal-title":"Clin Gastroenterol Hepatol"}],"container-title":["Digestive Diseases and Sciences"],"original-title":[],"language":"en","link":[{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/s10620-019-06009-3.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/article\/10.1007\/s10620-019-06009-3\/fulltext.html","content-type":"text\/html","content-version":"vor","intended-application":"text-mining"},{"URL":"http:\/\/link.springer.com\/content\/pdf\/10.1007\/s10620-019-06009-3.pdf","content-type":"application\/pdf","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2020,12,17]],"date-time":"2020-12-17T01:34:05Z","timestamp":1608168845000},"score":1,"resource":{"primary":{"URL":"http:\/\/link.springer.com\/10.1007\/s10620-019-06009-3"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2019,12,18]]},"references-count":26,"journal-issue":{"issue":"10","published-print":{"date-parts":[[2020,10]]}},"alternative-id":["6009"],"URL":"https:\/\/doi.org\/10.1007\/s10620-019-06009-3","relation":{},"ISSN":["0163-2116","1573-2568"],"issn-type":[{"value":"0163-2116","type":"print"},{"value":"1573-2568","type":"electronic"}],"subject":[],"published":{"date-parts":[[2019,12,18]]},"assertion":[{"value":"17 October 2019","order":1,"name":"received","label":"Received","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"10 December 2019","order":2,"name":"accepted","label":"Accepted","group":{"name":"ArticleHistory","label":"Article History"}},{"value":"18 December 2019","order":3,"name":"first_online","label":"First Online","group":{"name":"ArticleHistory","label":"Article History"}},{"order":1,"name":"Ethics","group":{"name":"EthicsHeading","label":"Compliance with Ethical Standards"}},{"value":"JMS declares consultancy: BMS, Boehringer Ingelheim, Galmed, Genfit, Gilead Sciences, Intercept Pharmaceuticals, IQVIA, MedImmune, Novartis, Pfizer, Roche. Research Funding: Gilead Sciences. Lectures: Abbvie, Falk Foundation, Takeda, MSD. All other authors declare no conflict of interest.","order":2,"name":"Ethics","group":{"name":"EthicsHeading","label":"Conflict of interest"}}]}}