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Factors that Influence Health-Related Quality of Life in Patients with Primary Sclerosing Cholangitis

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Abstract

Background

Primary sclerosing cholangitis (PSC) is an incurable, cholestatic liver disease often coincident with inflammatory bowel disease (IBD).

Aims

To evaluate the impact of liver disease and IBD on health-related quality of life (HRQoL) in PSC.

Methods

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.

Results

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.

Conclusions

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.

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Abbreviations

IBD:

Inflammatory bowel disease

HRQoL:

Health-related quality of life

PSC:

Primary sclerosing cholangitis

SF-36:

Short Form-36

PBC:

Primary biliary cirrhosis

UC:

Ulcerative colitis

CD:

Crohn’s disease

PHQ-9:

Patient Health Questionnaire-9

LDQOL:

Liver Disease Quality of Life Questionnaire

SIBDQ:

Short Inflammatory Bowel Disease Questionnaire

MRCP:

Magnetic resonance cholangiopancreatography

SSC:

Secondary sclerosing cholangitis

ALP:

Alkaline phosphatase

IQR:

Interquartile range

MCMC:

Markov chain Monte Carlo

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Acknowledgments

Thank you to all the patients who participated in this study. Dr. Hirschfield is supported by the NIHR Liver Biomedical Research Unit and is Principle Investigator for UK-PSC, a NIHR Translational Research Collaboration. The views expressed are those of the author(s) and not necessarily those of the National Health System, the National Institute for Health Research, or the Department of Health.

Funding

This study was funded by a grant awarded by PSC Partners Seeking a Cure.

Author information

Authors and Affiliations

  1. 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

    Angela C. Cheung, Harshna Patel & Javier Meza-Cardona

  2. Division of Gastroenterology, Toronto Western Hospital, University Health Network, University of Toronto, Toronto, ON, Canada

    Maria Cino

  3. Department of Psychiatry, Toronto General Hospital, University Health Network, University of Toronto, Toronto, ON, Canada

    Sanjeev Sockalingam

  4. Centre for Liver Research, Institute of Biomedical Research, Institute of Immunology and Immunotherapy, University of Birmingham, Birmingham, UK

    Gideon M. Hirschfield

Authors
  1. Angela C. Cheung
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  2. Harshna Patel
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  3. Javier Meza-Cardona
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  4. Maria Cino
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  5. Sanjeev Sockalingam
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  6. Gideon M. Hirschfield
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Corresponding author

Correspondence to Angela C. Cheung.

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Conflict of interest

Dr Gideon Hirschfield is a member of the PSC Partners Medical and Scientific Advisory Committee. The remaining authors have no conflicts of interest to declare.

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Cheung, A.C., Patel, H., Meza-Cardona, J. et al. Factors that Influence Health-Related Quality of Life in Patients with Primary Sclerosing Cholangitis. Dig Dis Sci 61, 1692–1699 (2016). https://doi.org/10.1007/s10620-015-4013-1

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  • DOI: https://doi.org/10.1007/s10620-015-4013-1

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