{"status":"ok","message-type":"work","message-version":"1.0.0","message":{"indexed":{"date-parts":[[2026,9,11]],"date-time":"2026-09-11T23:42:03Z","timestamp":1789170123819,"version":"build-2803163510"},"reference-count":27,"publisher":"Ovid Technologies (Wolters Kluwer Health)","issue":"1","license":[{"start":{"date-parts":[[2015,9,1]],"date-time":"2015-09-01T00:00:00Z","timestamp":1441065600000},"content-version":"tdm","delay-in-days":792,"URL":"http:\/\/doi.wiley.com\/10.1002\/tdm_license_1.1"}],"content-domain":{"domain":["lww.com","ovid.com"],"crossmark-restriction":true},"short-container-title":[],"published-print":{"date-parts":[[2013,7]]},"abstract":"<jats:title>Abstract<\/jats:title>\n                  <jats:sec>\n                    <jats:title\/>\n                    <jats:p>\n                      Primary biliary cirrhosis (PBC) has a complex clinical phenotype, with debate about the extent and specificity of frequently described systemic symptoms such as fatigue. The aim of this study was to use a national patient cohort of 2,353 patients recruited from all clinical centers in the UK to explore the impact of disease on perceived life quality. Clinical data regarding diagnosis, therapy, and biochemical status were collected and have been reported previously. Detailed symptom phenotyping using recognized and validated symptom assessment tools including the PBC-40 was also undertaken and is reported here. Perception of poor quality of life and impaired health status was common in PBC patients (35% and 46%, respectively) and more common than in an age-matched and sex-matched community control group (6% and 15%,\n                      <jats:italic toggle=\"yes\">P<\/jats:italic>\n                      &lt; 0.0001 for both). Fatigue and symptoms of social dysfunction were associated with impaired perceived quality of life using multivariate analysis. Fatigue was the symptom with the greatest impact. Depression was a significant factor, but appeared to be a manifestation of complex symptom burden rather than a primary event. Fatigue had its greatest impact on perceived quality of life when accompanied by symptoms of social dysfunction, suggesting that maintenance of social networks is critical for minimizing the impact of fatigue.\n                      <jats:italic toggle=\"yes\">Conclusion<\/jats:italic>\n                      : The symptom burden in PBC, which is unrelated to disease severity or ursodeoxycholic acid response, is significant and complex and results in significant quality of life deficit. The complexity of symptom burden, and its lack of relation to disease severity and treatment response, suggest that specific approaches to symptom management are warranted that address both symptom biology and social impact. (Hepatology 2013;58:-)\n                    <\/jats:p>\n                  <\/jats:sec>","DOI":"10.1002\/hep.26365","type":"journal-article","created":{"date-parts":[[2013,3,7]],"date-time":"2013-03-07T14:32:17Z","timestamp":1362666737000},"page":"273-283","update-policy":"https:\/\/doi.org\/10.1097\/lww.0000000000001000","source":"Crossref","is-referenced-by-count":208,"title":["Impact of primary biliary cirrhosis on perceived quality of life: The UK-PBC national study"],"prefix":"10.1097","volume":"58","author":[{"given":"George F.","family":"Mells","sequence":"first","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Greta","family":"Pells","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Julia L.","family":"Newton","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrew J.","family":"Bathgate","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Andrew K.","family":"Burroughs","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Michael A.","family":"Heneghan","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"James M.","family":"Neuberger","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Darren B.","family":"Day","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Samantha J.","family":"Ducker","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Richard N.","family":"Sandford","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"Graeme J.","family":"Alexander","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]},{"given":"David E.J.","family":"Jones","sequence":"additional","affiliation":[],"role":[{"vocabulary":"crossref","role":"author"}]}],"member":"276","reference":[{"key":"R1-31-20241201","doi-asserted-by":"crossref","first-page":"1552","DOI":"10.1016\/0016-5085(89)90526-X","article-title":"Prospective evaluation of oesophageal varicies in primary biliary cirrhosis: development, natural history and influence on survival.","volume":"96","author":"Gores","year":"1989","journal-title":"Gastroenterology"},{"key":"R2-31-20241201","doi-asserted-by":"crossref","first-page":"1138","DOI":"10.1002\/hep.510260508","article-title":"Hepatocellular carcinoma in primary biliary cirrhosis and its impact on outcomes.","volume":"26","author":"Jones","year":"1997","journal-title":"Hepatology"},{"key":"R3-31-20241201","doi-asserted-by":"crossref","first-page":"1261","DOI":"10.1056\/NEJMra043898","article-title":"Primary biliary cirrhosis.","volume":"353","author":"Kaplan","year":"2005","journal-title":"N Engl J Med"},{"key":"R4-31-20241201","doi-asserted-by":"crossref","first-page":"297","DOI":"10.1053\/j.gastro.2004.11.009","article-title":"The effect of ursodeoxycholic acid therapy on the natural history of primary biliary cirrhosis.","volume":"128","author":"Corpechot","year":"2005","journal-title":"Gastroenterology"},{"key":"R5-31-20241201","doi-asserted-by":"crossref","first-page":"715","DOI":"10.1053\/j.gastro.2005.12.029","article-title":"Excellent longterm survival in patients with primary biliary cirrhosis and biochemical response to ursodeoxycholic acid.","volume":"130","author":"Pares","year":"2006","journal-title":"Gastroenterology"},{"key":"R6-31-20241201","doi-asserted-by":"crossref","first-page":"237","DOI":"10.1016\/j.jhep.2009.04.009","article-title":"EASL practice guidelines on management of cholestatic liver diseases.","volume":"51","author":"Beuers","year":"2009","journal-title":"J Hepatol"},{"key":"R7-31-20241201","doi-asserted-by":"crossref","first-page":"1893","DOI":"10.1002\/hep.23238","article-title":"Bcell depletion with antiCD20 ameliorates autoimmune cholangitis but exacerbates colitis in transforming growth factorbeta receptor II dominant negative mice.","volume":"51","author":"Moritoki","year":"2009","journal-title":"Hepatology"},{"key":"R8-31-20241201","doi-asserted-by":"crossref","first-page":"199","DOI":"10.1136\/pmj.76.894.199","article-title":"Primary biliary cirrhosis: new perspectives in diagnosis and treatment.","volume":"76","author":"Prince","year":"2000","journal-title":"Postgrad Med J"},{"key":"R9-31-20241201","doi-asserted-by":"crossref","first-page":"2199","DOI":"10.1111\/j.1572-0241.2001.03956.x","article-title":"Healthrelated quality of life in chronic liver disease: the impact of type and severity of disease.","volume":"96","author":"Younossi","year":"2001","journal-title":"Am J Gastroenterol"},{"key":"R10-31-20241201","doi-asserted-by":"crossref","first-page":"489","DOI":"10.1002\/hep.20276","article-title":"Quality of life in patients with primary biliary cirrhosis.","volume":"40","author":"Poupon","year":"2004","journal-title":"Hepatology"},{"key":"R11-31-20241201","doi-asserted-by":"crossref","first-page":"760","DOI":"10.1111\/j.1572-0241.2000.01857.x","article-title":"Impact of fatigue on the quality of life in patients with primary biliary cirrhosis.","volume":"95","author":"Huet","year":"2000","journal-title":"Am J Gastroenterol"},{"key":"R12-31-20241201","doi-asserted-by":"crossref","first-page":"562","DOI":"10.1002\/hep.23683","article-title":"The specificity of fatigue in primary biliary cirrhosis: evaluation of a large clinic practice.","volume":"52","author":"AlHarthy","year":"2010","journal-title":"Hepatology"},{"key":"R13-31-20241201","doi-asserted-by":"crossref","first-page":"313","DOI":"10.1038\/nrgastro.2010.62","article-title":"Fatigue in primary biliary cirrhosis.","volume":"7","author":"Abbas","year":"2010","journal-title":"Nat Rev Gastrol Hepat"},{"key":"R14-31-20241201","doi-asserted-by":"crossref","first-page":"329","DOI":"10.1038\/ng.789","article-title":"Genomewide association study identifies 13 additional susceptibility loci for primary biliary cirrhosis.","volume":"43","author":"Mells","year":"2011","journal-title":"Nat Genet"},{"key":"R15-31-20241201","doi-asserted-by":"crossref","first-page":"1137","DOI":"10.1038\/ng.2395","article-title":"Dense finemapping study identifies novel disease loci and implicates coding and noncoding variation in primary biliary cirrhosis risk.","volume":"44","author":"Liu","year":"2012","journal-title":"Nat Genet"},{"key":"R16-31-20241201","doi-asserted-by":"crossref","first-page":"560","DOI":"10.1053\/j.gastro.2012.12.005","article-title":"Sex and age are determinants of the clinical phenotype of primary biliary cirrhosis and response to ursodeoxycholic acid.","volume":"144","author":"Carbone","year":"2013","journal-title":"Gastroenterology"},{"key":"R17-31-20241201","doi-asserted-by":"crossref","first-page":"1622","DOI":"10.1136\/gut.2005.065862","article-title":"Development, validation and evaluation of the PBC40, a disease specific health related quality of life measure for primary biliary cirrhosis.","volume":"54","author":"Jacoby","year":"2005","journal-title":"Gut"},{"key":"R18-31-20241201","doi-asserted-by":"crossref","first-page":"1496","DOI":"10.1002\/hep.21609","article-title":"The population prevalence and symptom associations of autonomic dysfunction in primary biliary cirrhosis.","volume":"45","author":"Newton","year":"2007","journal-title":"Hepatology"},{"key":"R19-31-20241201","doi-asserted-by":"crossref","first-page":"91","DOI":"10.1002\/hep.21230","article-title":"Fatigue in primary biliary cirrhosis is associated with excessive daytime somnolence.","volume":"44","author":"Newton","year":"2006","journal-title":"Hepatology"},{"key":"R20-31-20241201","doi-asserted-by":"crossref","first-page":"654","DOI":"10.1111\/j.1478-3231.2007.01500.x","article-title":"The role of psychological factors in the fatigue of primary biliary cirrhosis.","volume":"27","author":"Blackburn","year":"2007","journal-title":"Liver Int"},{"key":"R21-31-20241201","doi-asserted-by":"crossref","first-page":"871","DOI":"10.1002\/hep.22428","article-title":"Biochemical response to ursodeoxycholic acid and longterm prognosis in primary biliary cirrhosis.","volume":"48","author":"Corpechot","year":"2008","journal-title":"Hepatology"},{"key":"R22-31-20241201","doi-asserted-by":"crossref","first-page":"1099","DOI":"10.1016\/j.jhep.2007.01.036","article-title":"Depression in patients with primary biliary cirrhosis and primary sclerosing cholangitis.","volume":"46","author":"van Os","year":"2007","journal-title":"J Hepatol"},{"key":"R23-31-20241201","first-page":"546A","article-title":"Central cerebral activation is reduced in primary biliary cirrhosis and associated with excessive daytime somnolence.","volume":"46","author":"McDonald","year":"2007","journal-title":"Hepatology"},{"key":"R24-31-20241201","doi-asserted-by":"crossref","first-page":"541","DOI":"10.1002\/hep.22371","article-title":"Cognitive impairment in primary biliary cirrhosis: symptom impact and potential etiology.","volume":"48","author":"Newton","year":"2008","journal-title":"Hepatology"},{"key":"R25-31-20241201","doi-asserted-by":"crossref","first-page":"154","DOI":"10.1002\/hep.21003","article-title":"TNFalpha secreting monocytes are recruited into the brain of cholestatic mice.","volume":"43","author":"Kerfoot","year":"2006","journal-title":"Hepatology"},{"key":"R27-31-20241201","doi-asserted-by":"crossref","first-page":"2043","DOI":"10.1152\/japplphysiol.00195.2012","article-title":"Cardiac torsionstrain relationships in fatigued primary biliary cirrhosis patients show accelerated ageing: a pilot crosssectional study.","volume":"112","author":"Hollingsworth","year":"2012","journal-title":"J Appl Physiol"},{"key":"R28-31-20241201","doi-asserted-by":"crossref","first-page":"1095","DOI":"10.1016\/j.jhep.2010.05.036","article-title":"Central nervous system dysfunction in primary biliary cirrhosis patients and its relationship to symptoms.","volume":"53","author":"McDonald","year":"2010","journal-title":"J Hepatol"}],"container-title":["Hepatology"],"original-title":[],"language":"en","link":[{"URL":"https:\/\/api.wiley.com\/onlinelibrary\/tdm\/v1\/articles\/10.1002%2Fhep.26365","content-type":"unspecified","content-version":"vor","intended-application":"text-mining"},{"URL":"https:\/\/journals.lww.com\/01515467-201307000-00031","content-type":"unspecified","content-version":"vor","intended-application":"similarity-checking"}],"deposited":{"date-parts":[[2024,12,1]],"date-time":"2024-12-01T09:14:17Z","timestamp":1733044457000},"score":1,"resource":{"primary":{"URL":"https:\/\/journals.lww.com\/01515467-201307000-00031"}},"subtitle":[],"short-title":[],"issued":{"date-parts":[[2013,7]]},"references-count":27,"journal-issue":{"issue":"1","published-print":{"date-parts":[[2013]]}},"URL":"https:\/\/doi.org\/10.1002\/hep.26365","relation":{},"ISSN":["0270-9139"],"issn-type":[{"value":"0270-9139","type":"print"}],"subject":[],"published":{"date-parts":[[2013,7]]}}}