title: Factors that Influence Health-Related Quality of Life in Patients with Primary Sclerosing Cholangitis.
abstract: Primary sclerosing cholangitis (PSC) is an incurable, cholestatic liver disease often coincident with inflammatory bowel disease (IBD).
  
  
  
  To evaluate the impact of liver disease and IBD on health-related quality of life (HRQoL) in PSC.
  
  
  
  A mixed-methods, cross-sectional study was performed at a tertiary center. Short Form-36 (SF-36) scores were compared between PSC, Canadian normative data, and disease controls. Disease-specific instruments scores [PBC-40, Short IBD questionnaire, Liver Disease Quality of Life Questionnaire (LDQOL)] were compared between PSC and disease controls. Multivariable regression identified factors independently associated with final SF-36 component scores. Qualitative evaluation of patient questionnaires was performed using a content analysis framework.
  
  
  
  One hundred and sixty-two surveys were completed (99 PSC, 26 primary biliary cirrhosis, 16 non-autoimmune cholestatic liver disease, and 21 IBD). PSC patients had significantly lower SF-36 scores than Canadian controls, but similar scores to disease controls. LDQOL most accurately predicted HRQoL. Factors negatively associated with physical HRQoL included shorter IBD duration, liver disease symptoms, and decompensated cirrhosis. Mental HRQoL was influenced by liver disease and IBD symptoms, pruritus, social isolation, and depression. Nearly 75 % expressed existential anxiety regarding disease progression and diminished life expectancy, with 25 % disclosing social isolation.
  
  
  
  Patients with PSC have significantly lower HRQoL than healthy controls. Both symptoms of IBD and chronic liver disease impact HRQoL in patients with PSC, which lead to significant psychologic burden that is expressed by existential anxieties and social isolation. A PSC-specific HRQoL tool is critical to adequately quantify the distinct impact of IBD and cholestatic liver disease.
authors.affiliation: Division of Gastroenterology, Department of Medicine, Toronto Centre for Liver Disease, University of Toronto, 200 Elizabeth Street, Norman Urquhart Wing, 13th Floor, Toronto, ON, M5G 2C4, Canada. AngelaC.Cheung@uhn.ca.
authors.forename: Angela C
authors.initials: AC
authors.lastname: Cheung
authors.affiliation: Division of Gastroenterology, Department of Medicine, Toronto Centre for Liver Disease, University of Toronto, 200 Elizabeth Street, Norman Urquhart Wing, 13th Floor, Toronto, ON, M5G 2C4, Canada.
authors.forename: Harshna
authors.initials: H
authors.lastname: Patel
authors.affiliation: Division of Gastroenterology, Department of Medicine, Toronto Centre for Liver Disease, University of Toronto, 200 Elizabeth Street, Norman Urquhart Wing, 13th Floor, Toronto, ON, M5G 2C4, Canada.
authors.forename: Javier
authors.initials: J
authors.lastname: Meza-Cardona
authors.affiliation: Division of Gastroenterology, Toronto Western Hospital, University Health Network, University of Toronto, Toronto, ON, Canada.
authors.forename: Maria
authors.initials: M
authors.lastname: Cino
authors.affiliation: Department of Psychiatry, Toronto General Hospital, University Health Network, University of Toronto, Toronto, ON, Canada.
authors.forename: Sanjeev
authors.initials: S
authors.lastname: Sockalingam
authors.affiliation: Centre for Liver Research, Institute of Biomedical Research, Institute of Immunology and Immunotherapy, University of Birmingham, Birmingham, UK.
authors.forename: Gideon M
authors.initials: GM
authors.lastname: Hirschfield
mesh_terms: D000328:Adult; D000368:Aged; D015209:Cholangitis, Sclerosing / Q000473:pathology*; D003625:Data Collection; D005260:Female; D006801:Humans; D008297:Male; D008875:Middle Aged; D011788:Quality of Life; D011795:Surveys and Questionnaires
keywords: Cholestatic liver disease; Health-related quality of life; Qualitative research; Short Form-36