Impact of primary biliary cirrhosis on perceived quality of life: The UK-PBC national study
Open accessAbstract
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%, P < 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. Conclusion : 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:-)
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Symptoms associated with PBC reduce quality of life and include cholestatic pruritus, sicca complex, abdominal discomfort, and fatigue.
“The symptoms associated with PBC impact on QoL, and include: cholestatic pruritus, sicca complex, abdominal discomfort and fatigue [29,30].”
From EASL Clinical Practice Guidelines: The diagnosis and management of patients with primary biliary cholangitis. (2017) · cites this paper as [29]
The Discussion reports that PBC symptoms can substantially impair quality of life, with fatigue having the greatest absolute impact; the Abstract identifies cholestatic pruritus as a PBC symptom. Our copy does not identify sicca complex or abdominal discomfort among the symptoms studied, so it does not support those parts of the list (searched: sicca, dry eyes/dry mouth, abdominal discomfort/pain). (LLM, conf=0.85)
Experimental — accuracy not yet measuredFatigued patients with PBC have lower levels of social engagement.
“Fatigued patients perceive a poor QoL compared to controls, and their levels of social engagement are lower [29,30].”
From EASL Clinical Practice Guidelines: The diagnosis and management of patients with primary biliary cholangitis. (2017) · cites this paper as [29]
The Results report that 89% of fatigued PBC patients with poor quality of life had significant social impairment. But only 11% of fatigued patients whose quality of life remained good had social impairment, so the claim overgeneralizes the finding to fatigued patients as a whole. (LLM, conf=0.87)
Experimental — accuracy not yet measuredFatigue is not specific to PBC, is frequently reported by patients (over 50%) and when severe (20%) is a significant cause of QoL impairment.
“Although fatigue is not specific to PBC, it is frequently reported by patients (over 50%) and when severe, as it is in 20% of patients, is a significant cause of QoL impairment.12 171 205–208”
From The British Society of Gastroenterology/UK-PBC primary biliary cholangitis treatment and management guidelines (2018) · cites this paper as [171]
The Discussion (page unavailable) describes fatigue as a major burden and strongly associated with quality of life, while noting that some patients with significant fatigue still reported good quality of life. The Results (page unavailable) identify a severely fatigued subgroup, but the retrieved passages do not establish that over 50% of patients report fatigue or that 20% have severe fatigue. They also do not establish the claim that fatigue is not specific to PBC. (LLM, conf=0.85)
Experimental — accuracy not yet measuredFatigue has been shown to be the symptom with the biggest impact on patients; fatigued patients perceive a poor QoL compared with controls and their levels of social engagement are lower.
“Fatigue has been shown to be the symptom with the biggest impact on patients. Fatigued patients perceive a poor QoL compared with controls and their levels of social engagement are lower.171 219”
From The British Society of Gastroenterology/UK-PBC primary biliary cholangitis treatment and management guidelines (2018) · cites this paper as [171]
The Results report that fatigue had the greatest symptom impact in PBC and that fatigued patients with poor quality of life often had social impairment. But the comparison is between groups of fatigued PBC patients, not between their perceived quality of life and that of controls; nearly 300 fatigued patients retained good quality of life. The Discussion likewise describes fatigue as a risk for impaired quality of life whose impact varies with social functioning. (LLM, conf=0.88)
Experimental — accuracy not yet measuredPBC symptoms substantially affect quality of life and can be broad.
“The symptoms associated with PBC have a significant impact on QoL for patients [29], and can be broad.”
From EASL Clinical Practice Guidelines: The diagnosis and management of patients with primary biliary cholangitis. (2017) · cites this paper as [29]
Fatigued patients with PBC perceive a poorer quality of life than controls.
“Fatigued patients perceive a poor QoL compared to controls, and their levels of social engagement are lower [29,30].”
From EASL Clinical Practice Guidelines: The diagnosis and management of patients with primary biliary cholangitis. (2017) · cites this paper as [29]
The symptoms of PBC significantly impair quality of life and do not typically improve with UDCA or OCA treatment.
“The symptoms of PBC significantly impair quality of life (182) and do not typically improve with UDCA or OCA treatment.”
From Primary Biliary Cholangitis: 2018 Practice Guidance from the American Association for the Study of Liver Diseases (2019) · cites this paper as [182]
The symptoms associated with PBC are important and have a significant impact on life quality for patients.
“The symptoms associated with PBC are important and have a significant impact on life quality for patients.171”
From The British Society of Gastroenterology/UK-PBC primary biliary cholangitis treatment and management guidelines (2018) · cites this paper as [171]
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- Authors
- Mells, G. F., Pells, G., Newton, J. L., Bathgate, A. J., Burroughs, A. K., Heneghan, M. A., Neuberger, J. M., Day, D. B., Ducker, S. J., Sandford, R. N., Alexander, G. J., & Jones, D. E. J.
- Affiliations
- University of Cambridge (GB)
- Newcastle University (GB)
- NIHR Newcastle Biomedical Research Centre (GB)
- Edinburgh Royal Infirmary (GB)
- The Royal Free Hospital (GB)
- King's College Hospital NHS Foundation Trust (GB)
- Queen Elizabeth Hospital Birmingham (GB)
- Corresponding author
- Jones, David E.J.
- ORCID
- Venue
- Hepatology
- Published
- 2013-07
- Volume
- 58
- Issue
- 1
- Pages
- 273-283
- ISSN
- 0270-9139 (ISSN-L)
- OpenAlex
- W2093292173
- Semantic Scholar
- c3f7cee219b4024ce1b44b26b30d2b5f4b77d7e4
- Cited by
- 227
- References
- 29
- Citations by year
- 2026: 27 · 2025: 27 · 2024: 16 · 2023: 17 · 2022: 15 · 2021: 19 · 2020: 15 · 2019: 22 · 2018: 22 · 2017: 12 · 2016: 9 · 2015: 15 · 2014: 8 · 2013: 2 · 2012: 1
- FWCI
- 7.07
- Type
- journal article
- Language
- en
- Open access
- bronze
- doi.org
- Locations
- Hepatology (publishedVersion)
- PubMed (publishedVersion)
- Research Portal (King's College London) (publishedVersion, other-oa)
- Newcastle University ePrints (Newcastle Univesity)
- Funders
- Royal Devon and Exeter NHS Foundation Trust
- Wellcome Trust
- NHS Greater Glasgow and Clyde
- Sandwell and West Birmingham Hospitals NHS Trust
- Nottingham University Hospitals NHS Trust
- University Hospitals Bristol NHS Foundation Trust
- University Hospitals of Leicester NHS Trust
- Heart of England NHS Foundation Trust
- Royal Free London NHS Foundation Trust
- University Hospitals Plymouth NHS Trust
- National Institute for Health and Care Research (IS-BRC-0211-10046)
- University of Cambridge
- University of Southampton
- Imperial College London
- Imperial College Healthcare NHS Trust
- University College London
- Chelsea and Westminster Hospital NHS Foundation Trust
- Newcastle University
- University of Nottingham
- Cambridge University Hospitals
- Newcastle upon Tyne Hospitals NHS Foundation Trust
- Isaac Newton Trust
- North Bristol NHS Trust
- University College London Hospitals NHS Foundation Trust
- Norfolk and Norwich University Hospitals NHS Foundation Trust
- Sheffield Teaching Hospitals NHS Foundation Trust
- University Hospitals Coventry and Warwickshire NHS Trust
- Medical Research Council (MR/L001489/1, MR/K501037/1)
- Guy's and St Thomas' NHS Foundation Trust
- NIHR Newcastle Biomedical Research Centre (BH121729)
- Topics
- Health Sciences › Medicine › Hepatology › Liver Diseases and Immunity
- Health Sciences › Medicine › Surgery › Pediatric Hepatobiliary Diseases and Treatments
- Health Sciences › Medicine › Hepatology › Liver Disease and Transplantation
- Keywords
- primary biliary cirrhosis, quality of life, fatigue, social dysfunction, depression, symptom burden, health-related quality of life, symptom management, social networks
- MeSH terms
- Cross-Sectional Studies, Depression, Fatigue, Female, Humans, Liver Cirrhosis, Biliary, Male, Perception, Quality of Life, Surveys and Questionnaires, Cohort Studies
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