Perceived Stigma in Patients with Autoimmune Hepatitis.

Perceived stigma (PS) adversely impacts psychosocial and disease outcomes in patients with chronic liver diseases (CLD), and those with autoimmune hepatitis (AIH) may be at risk for PS given inaccurate assumptions about the origin of their diagnosis. The aims of the current study are to describe the frequency of PS in patients with AIH, compare rates of PS in AIH to rates of PS in primary biliary cholangitis (PBC) and CLD, and examine demographic correlates of PS. 262 adults with AIH (95% female, Mage = 51.53 years) completed online questionnaires on demographics, disease information, and PS. 54-68% reported PS with themes of selective disclosure, non-disclosure, or hiding diagnosis. PS was higher in those with AIH compared to those with PBC, but lower than those with various CLD. Age was inversely related to PS. Given the results, provider screening of PS and integration of clinical health psychologists may be helpful for identifying PS in patients with AIH.

Quality of Life in Patients with Primary Biliary Cholangitis: A Single-Center Experience in Serbia.

BACKGROUND: Primary biliary cholangitis (PBC) is a chronic cholestatic autoimmune disease, predominantly affecting middle-aged women, which may progress to end-stage liver disease. We aimed to assess the quality of life (QoL) in patients with PBC given that social, economic, and geographical factor also influence QoL. METHODS: This study included patients with diagnosed PBC according to the European Association for the Study of the Liver guidelines, who were treated for at least 6 months in order to allow adequate time for the initial burden of symptoms to subside. We used the PBC-40 questionnaire validated in the Serbian language. RESULTS: The mean total PBC-40 score was 89.4 ± 29.3. The overall frequency of moderate and severe involvement in each domain was as follows: 84.9% (n = 107) in "Symptoms," 29.3% (n = 36) in "Itch," 76.4% (n = 97) in "Fatigue," 58.1% (n = 72) in "Cognitive," 77.2% (n = 98) in "Social," and 70.9% (n = 90) in "Emotional." There was a statistically significant negative correlation of disease duration and albumin score with the Emotional domain score. Furthermore, this domain showed a significant positive correlation with the Mayo score. CONCLUSION: The present study demonstrates that patients with PBC have significant impairment in QoL with fatigue being the most prevalent symptom. The Social and Emotional domains were also significantly affected in these individuals, particularly in patients with peripheral edema who exhibited worse QoL that those who were euvolemic.

Neuropsychiatric symptoms in hepatitis C patients resemble those of patients with autoimmune liver disease but are different from those in hepatitis B patients.

Chronic fatigue, mood alterations and cognitive impairment are frequent accessory symptoms of HCV infection. Fatigue and mood alterations have also been observed in autoimmune hepatitis (AIH) and primary biliary cholangitis (PBC), but not in hepatitis B virus (HBV)-infection, thus indicating an autoimmune response as possible cause of HCV infection-associated encephalopathy. Data, however, are sparse. This study aimed to prove that HCV patients feature similar to those with autoimmune liver disease but contrary to HBV patients regarding neuropsychiatric symptoms. A total of 132 noncirrhotic patients (HCV: 46, HBV: 22, AIH: 27, PBC: 29, AIH/PBC: 8) completed questionnaires addressing the domains mentioned above. Eighty-eight underwent a comprehensive neuropsychological assessment. Patient groups were compared among each other and to 33 healthy controls. Fatigue, anxiety and depression scores were significantly increased, and the SF-36 mental score significantly decreased in all patient groups compared to controls. Fatigue was significantly more pronounced in HCV than in HBV patients. HCV patients scored significantly worse than HBV patients but not AIH and PBC patients in the SF-36. HCV, AIH and PBC but not HBV patients did significantly worse than controls in word learning. Recognition of words was impaired in HCV, AIH and PBC patients and recognition of figures in HCV patients, exclusively (P ≤ 0.002). HCV patients did also worse than controls and HBV patients concerning alertness and working memory (P ≤ 0.001). The neuropsychiatric profiles of HCV patients are similar to those of AIH and PBC patients but differ from those of HBV patients, suggesting an autoimmune response as a possible cause for these differences.

Activation of TRPV1 receptors affects memory function and hippocampal TRPV1 and CREB mRNA expression in a rat model of biliary cirrhosis.

OBJECTIVE: Memory impairment induced by biliary cirrhosis is associated with abnormalities in the function of different neurotransmitter systems. However, the exact molecular mechanisms involved in the learning and memory dysfunctions following biliary cirrhosis is largely unknown. This study set out to determine whether activation of transient receptor potential vanilloid type 1 (TRPV1) in the CA1 area of the hippocampus in rats improve memory impairment induced by biliary cirrhosis. METHODS: To assess learning and memory, passive avoidance task was carried out using a shuttle box. The mRNA expression of TRPV1 and cAMP response element binding (CREB) protein in the hippocampus were also evaluated by qT-PCR. RESULTS: Our results indicated that activation of TRPV1 channels by capsaicin significantly decreased memory impairment and increased mRNA expression of the TRPV1 and CREB in the hippocampus of rats with biliary cirrhosis. Our findings also demonstrated that a positive correlation existed between mRNA expression of TRPV1 and CREB, and between memory function and TRPV1 expression. DISCUSSION: Taken together, the results of this study support the view that TRPV1 receptor may play an important role in the regulation of learning and memory functions, and suggest that activation of TRPV1 channels seems to be a promising therapeutic target for learning and memory impairments following biliary cirrhosis.

Assessment of health related quality of life in polish patients with primary biliary cirrhosis.

BACKGROUND: Most patients with primary biliary cirrhosis (PBC) have impaired health related quality of life (HRQoL), as assessed by PBC-specific HRQoL (PBC-40) and generic (SF-36) questionnaires. Data on the applicability of PBC-27, a shorter version of PBC-40, have been limited. AIMS: To assess HRQoL in Polish PBC patients, applying PBC-40, PBC-27 and SF-36 and to associate clinical or laboratory parameters with HRQoL factors. METHODS: A total of 205 PBC patients (188 females) were analyzed using PBC-40, PBC-27 and SF-36; 85 disease-free demographically matched (in terms of age, gender, ethnicity) individuals were used as normal controls. RESULTS: When compared to controls, PBC patients had significantly impaired HRQoL across all the domains of SF-36. HRQoL impairment by PBC-40 and PBC-27 was comparable between cirrhotics and non-cirrhotics, except for significantly worse Itch in cirrhotics (6.5±4.9 vs 5.1±4.3; P=0.03). In PBC-40/27, alkaline phosphatase (ALP) levels correlated with itch (P=0.0003). Female patients had marginally impaired cognitive function compared to males by PBC-40 (P=0.06). Other gender-related differences were not found. Anti-gp210 positive, as well as AMA negative PBC patients, had worse HRQoL features in itch and social/emotional domains of PBC-40/PBC-27 questionnaires. Very strong correlations (P<0.0001) between PBC-40/PBC-27 and SF-36 were seen for several domains. CONCLUSIONS: HRQoL is significantly impaired in Polish patients with PBC, independently of gender and disease severity. PBC-40 and PBC-27 questionnaires are efficient in detecting HRQoL impairment in Polish PBC patients. The striking correlation between PBC-40/PBC-27 and SF-36 confirms the usefulness of the former HRQoL measures in PBC patients from Central-Eastern Europe.

Quality of life and illness perception in primary biliary cirrhosis: a controlled cross-sectional study.

OBJECTIVE: The aim of this study was to understand better the quality of life (QOL) and illness perception in women with primary biliary cirrhosis (PBC) through a comparison with women having diabetes. METHODS: One hundred and ninety-four women took part in this study: 130 with PBC, 64 with type 2 diabetes. They were administered the SF-12 to measure QOL and the Brief Illness Perception Questionnaire to assess representations of their illness. Analysis of covariance with bootstrapping was used to compare QOL and illness perception scores by controlling age and mean disease duration. RESULTS: Physical QOL was significantly worse for women with PBC than for women with diabetes. Women with PBC felt their disease would last longer and reported more symptoms and concerns related to their disease than women with diabetes. Significant differences were also observed for causes: women with PBC mainly reported autoimmune, emotional, unknown/unlucky and medical causes whereas women with diabetes reported mostly lifestyle and hereditary causes. Marginally significant differences were observed regarding consequences on daily life, feeling of control over the disease and emotional responses, which were shown to be worse in PBC. Mental QOL, treatment control and overall understanding of the disease was similar in both groups. CONCLUSIONS: This study shows that women with PBC have a worse QOL and somewhat different illness perception than women with diabetes. Further research could help understand PBC specificities better in order to improve patient care, especially if factors such as fatigue or rarity of the disease explain these results.

TRAF1-C5 affects quality of life in patients with primary biliary cirrhosis.

BACKGROUND: Previous studies reported associations between specific alleles of non-HLA immunoregulatory genes and higher fatigue scores in patients with primary biliary cirrhosis (PBC). AIM: To study the relationship between variables of health-related quality of life (HRQoL) and single nucleotide polymorphisms of TRAF1-C5, a member of the tumor necrosis factor receptor family. PATIENTS AND METHODS: TRAF1-C5 gene polymorphisms, rs2900180 and rs3761847, were analysed in 120 Caucasian PBCs. The HRQoL was assessed with SF-36, PBC-40, and PBC-27 questionnaires. RESULTS: We found a negative association between TT genotype of rs2900180 and SF-36's domains vitality (P < 0.05), mental health (P < 0.05), and mental component summary score (P < 0.05). GG homozygotes of rs3761847 had lower vitality (P < 0.05), mental health (P < 0.05), mental component summary score (P < 0.05) and impairment of social functioning (P < 0.01). Allelic analysis has shown that T allele of rs2900180 and G allele of rs3761847 related to SF-36's vitality (P < 0.05 and P < 0.01), social functioning (P < 0.05 and P < 0.05), mental health (P < 0.01 and P < 0.05), and mental component summary score (P < 0.01 and P < 0.05), respectively. Genotyping and allelic analysis did not reveal correlation with PBC-40 and PBC-27 domains. CONCLUSION: The association between rs2900180 and rs3761847 polymorphisms and HRQoL variables indicates that TRAF1 is involved in the induction of impaired QoL in PBC.

Impact of primary biliary cirrhosis on perceived quality of life: the UK-PBC national study.

UNLABELLED: 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.

'It's as if PBC didn't exist': the illness experience of women affected by primary biliary cirrhosis.

This study investigates the illness experience of women who are affected by primary biliary cirrhosis (PBC), a rare chronic liver disease which mostly affects women. Despite the fact that PBC is medically recognised, it shares many characteristics with other chronic and controversial conditions which may lead to delegitimation of the patient's experience of illness, impacting on the construction of the patient's self-identity. Twenty three women took part in semi-structured interviews. Data were analysed using interpretative phenomenological analysis and the analysis identified three themes that are interrelated and characterised by a dynamic of normalisation. The first theme, the delegitimation of women's experience, implies denial of patients' sick-role, trivialisation of fatigue and lack of consideration of patients' needs. The second theme, PBC as a challenge to women's social identity refers to how patients face delegitimation and manage the illness's consequences for relationships and social roles. The third theme, the need for biographical continuity, focuses on how patients construct their ill identities as a consequence of both the delegitimation and the challenges posed by PBC. Results are discussed in a gender perspective, highlighting how delegitimation and identity construction processes are influenced by women's social roles and conditions.

Uncertainty and liver transplantation: women with primary biliary cirrhosis before and after transplant.

Uncertainty is a frequent feature of chronic illness and can have a particularly important impact in the case of organ transplantation. This study of 100 women with primary biliary cirrhosis who were either waiting for or had already had a liver transplant focused on both changes in uncertainty with transplant and the correlates of uncertainty both pre- and post-transplant. While those who were post-transplant had significantly lower uncertainty scores (measured by the Mishel Uncertainty in Illness Scale-Adult Version-MUIS-A) than those on the waiting list, uncertainty was still persistent and associated with a reduced quality of life. The most significant factors in relation to uncertainty were fatigue, depression, anxiety, and dissatisfaction with medical information received. It is important for both patients and transplant team members to recognize the impact of uncertainty on a patient's well-being, both before and after a transplant, and to address the underlying factors that continue to compromise quality of life even after a life-saving procedure.

The independent effects of fatigue and UDCA therapy on mortality in primary biliary cirrhosis: results of a 9 year follow-up.

BACKGROUND & AIMS: Long-term outcome in primary biliary cirrhosis (PBC) remains unclear. Whilst response to ursodeoxycholic acid (UDCA) is associated with good outcome, this effect is not universal. Early data from our group have suggested that one factor associated with a poorer outcome in PBC is fatigue. The aim of this study was to explore the inter-relationship between UDCA use, response, and fatigue in determining outcome over 9 years in a unique, comprehensive patient cohort. METHODS: Longitudinal prospective study of a geographically-defined complete cohort of PBC patients in North-East England and matched community controls. RESULTS: Survival to death or transplant was significantly lower in PBC patients than in the case-control population (88/136 (65%) v 114/136 84% (p<0.001 by log-rank test), with better survival in UDCA responders (defined using the Paris criteria) than in patients not treated with UDCA at study outset. Compared to the whole control group survival was reduced in PBC patients fatigued at study outset but not in those without fatigue (p<0.0001); an effect independent of the beneficial effect of UDCA response and of conventional parameters of liver disease severity. UDCA responders without fatigue at the study outset had a 9 year survival which was identical to controls. Patients without fatigue at the study outset who developed fatigue during follow-up had significantly worse survival than patients who remained without fatigue throughout (p<0.05). Fatigued controls had worse survival than non-fatigued controls (p = 0.05). CONCLUSIONS: Survival in a comprehensive cohort of PBC patients is substantially reduced compared with case-matched community controls. Development of fatigue and non-treatment with UDCA were specifically (and independently) associated with increased risk of death in PBC.

Impaired cerebral autoregulation in primary biliary cirrhosis: implications for the pathogenesis of cognitive decline.

BACKGROUND: Cognitive impairment is recognised in the early stages of primary biliary cirrhosis (PBC). AIMS: To determine the mechanisms that underlie the cognitive dysfunction that can occur in early-stage PBC, with a particular focus on the role of autonomic dysfunction and altered cerebral autoregulation. PATIENTS: Early-stage PBC patients, and age- and sex-matched controls. INTERVENTIONS AND MAIN OUTCOME MEASURES: Brain magnetic resonance imaging to determine the relationship between structural brain abnormalities (T(2)) and cerebral vasculature responsiveness assessed using the Valsalva manoeuvre. Dynamic assessment of cerebral vascular flow using transcranial Doppler was also performed in PBC subjects to derive the pulsatility index (a marker of cerebral resistance) and the autoregulatory slope index [ASI; ratio between the cerebral blood flow velocity and blood pressure (BP)]. RESULTS: Cerebral resistance was increased (P=0.04), and cerebral autoregulation in response to the Valsalva was significantly impaired in the PBC group with markedly lower mean ASI values compared with the controls (7.8+/-7.0 vs -8.5+/-8.4; P=0.002). All controls had normal cerebral autoregulation compared with only 20% of the PBC group. Indicators of sympathetic failure (BP change between Valsalva phases III-IV and low-frequency heart rate variability) correlated with increasing globus pallidus (GP) T(2) values (P<0.05), beyond the effect of age. CONCLUSION: This study demonstrates the presence of increased cerebral vascular resistance and abnormal cerebral autoregulation in PBC patients, and identifies a potentially important association between the degree of abnormality in structural changes in the GP. These findings suggest that organic brain injury in PBC is directly related to autonomic dysfunction.

A short version of a HRQoL questionnaire for Italian and Japanese patients with Primary Biliary Cirrhosis.

BACKGROUND: The available self-report questionnaire for the quality of life in patients with primary biliary cirrhosis (PBC-40) is currently validated only in the British population but it lacks an evaluation of its dimensionality. AIMS: To validate the Italian and Japanese versions of PBC-40 and to assess the dimensionality of the original structure of PBC-40 by a confirmatory factor analysis. PBC-40 was translated to Italian and Japanese using the forward-backward method and then reviewed in focus groups in the framework of a large multicentric study. METHODS: A sample of 290 patients with PBC (125 Italian and 165 Japanese) was administered two questionnaires previously validated for PBC-specific (PBC-40) and general quality of life (SF-36). RESULTS: The confirmatory model failed to fit adequately the original hypothesized structure. A principal component analysis led to a seven-factor structure, with exclusion of 13 items characterized by lower load; PBC-27 questionnaire was the final instrument. The validity of the PBC-27 was supported by its strong correlation with the SF-36 scores. CONCLUSION: We here propose an alternative structure of the quality of life questionnaire for PBC, namely PBC-27, which appears to be effective in detecting the impact of PBC on quality of life in Italian and Japanese patients.

Cognitive impairment in primary biliary cirrhosis: symptom impact and potential etiology.

UNLABELLED: Qualitative studies suggest that patients with primary biliary cirrhosis (PBC) experience significant problems with memory and concentration. Studies of nonhepatic disease have linked hypotension and cognitive impairment. In this study, we determined the prevalence of cognitive symptoms in PBC, examined the relationship between symptoms and overt cognitive impairment and structural brain lesions, and explored the role of autonomic dysfunction. The prevalence of cognitive symptoms was determined in 198 patients with PBC. Twenty-eight representative early-stage female patients with PBC and 11 matched controls underwent formal cognitive testing at baseline and after 2 years of follow-up. Autonomic nervous system function was assessed according to heart rate variability and baroreflex sensitivity. Eleven subjects with PBC had structural brain lesions quantified via magnetic resonance imaging. Cognitive symptoms were frequent in our PBC population, with 53% of patients experiencing moderate or severe problems with concentration and/or memory, which were unrelated in their severity to biochemical and histological makers of liver disease severity, suggesting that this symptom burden is largely or entirely unrelated to hepatic encephalopathy. Perceived cognitive symptoms correlated with objectively assessed cognitive impairment (r(2) = 0.2, P < 0.05). Cognitive deficits were seen in the PBC cohort compared with controls, with significant decline detected over 2 years of follow-up. Correlations were seen between cognitive performance (full-scale intelligence quotient) and systolic blood pressure (P = 0.01, r(2) = 0.2) with decline in cognitive function associated with autonomic abnormalities. Structural brain lesions were found in PBC, the density of which correlated with degree of cognitive impairment (P = 0.01, r(2) = 0.5) and autonomic function (P = 0.03, r(2) = 0.2). CONCLUSION: Cognitive symptoms are prevalent in PBC independent of liver disease severity and are associated with poorer performance on objective cognitive testing. Cognitive impairment is, in turn, associated with structural brain lesions and autonomic dysfunction, which may predict risk of cognitive decline.

Fatigue as a major predictor of quality of life in women with autoimmune liver disease: the case of primary biliary cirrhosis.

OBJECTIVES: Fatigue is a nearly universal symptom of many chronic diseases, yet it is often poorly understood and underappreciated as a factor in quality of life (QOL). Generally, clinicians have relied on subjective measures of fatigue, if they consider it at all. This study uses well-validated instruments to examine fatigue as a predictor of QOL in women with primary biliary cirrhosis (PBC), an autoimmune, chronic liver disease. METHODS: Eighty-one women with PBC completed a survey that included measures of fatigue (Fatigue Impact Scale) and QOL (SF-36) as well as demographic variables (age, education) and medical information (symptoms, stage of illness, time since diagnosis). QOL results for the sample were compared with those of a nationally normed U.S. population. Bivariate and multivariate analyses were conducted to identify contributors to variation in QOL. RESULTS: Compared with national norms, QOL for this PBC population was significantly impaired. When all variables with bivariate significance in relation to QOL were included in multivariate analyses, results showed fatigue to be the primary predictor of QOL, including all 8 QOL scales and the 2 summary scales. Regression results, dominated by fatigue, explain 25-59% of the variance in QOL. CONCLUSIONS: Fatigue has profound effects on every aspect of life for women with PBC-physical, social, emotional, and psychological. The results lead to recommendations for health care providers to assess fatigue in their patients with PBC and to take steps, where warranted, to mitigate its effects.

An integrated care pathway improves quality of life in Primary Biliary Cirrhosis.

BACKGROUND: Clinical management of the chronic autoimmune liver disease, Primary Biliary Cirrhosis (PBC) involves addressing the underlying liver disease and a range of symptoms independent of liver disease severity. We have formally explored how these two perspectives of chronic disease management can be combined into a clinic consultation and impact upon quality of life (QOL) in PBC. AIMS: To develop and implement the first Integrated Care Pathway (ICP) for the management of liver disease progression and symptom management in PBC. METHODS: Process mapping of current practice by a multidisciplinary group developed a flowchart of care from which the clinical record evolved. Symptom assessment is incorporated into the PBC ICP (QOL; PBC-40, autonomic symptoms; Orthostatic Grading Scale, daytime sleepiness; Epworth Sleepiness Scale). All patients were considered who attended clinic between July 2005 and June 2006. Symptom assessment was repeated after 1 year in those participating in the initial clinic cohort. RESULTS: The PBC ICP was successfully introduced into our clinical environment with high levels of patient satisfaction. A total of 225 PBC patients attended over 12 months. Initial QOL assessments were in 195 (87%). Five patients died (3%). Repeat assessment 1 year later occurred in 149 subjects (149/190; 78%). All symptom domains improved after ICP implementation with significant improvements in those with moderate and severe symptoms in all PBC-40 symptom domains (P < 0.02). In those with severe fatigue (n = 38) symptom improvement was even more dramatic (P = 0.002). CONCLUSION: ICP implementation delivers evidence-based care, leads to improvements in QOL coupled with high levels of patient satisfaction.

Health-related quality of life in patients with chronic hepatitis B.

UNLABELLED: Although chronic hepatitis C (CH-C) has consistently been shown to impair patients' health-related quality of life (HRQL), the impact of chronic hepatitis B (CH-B) on HRQL has not been fully explored. AIM: Compare HRQL between patients with CH-B, CH-C, primary biliary cirrhosis (PBC) and healthy controls. DESIGN: Three HRQL questionnaires [Chronic Liver Disease Questionnaire (CLDQ), Short Form 36 (SF-36) and the Health Utility Index (HUI Mark-2 and Mark-3)] were administered prospectively. Additional clinical and laboratory data and normative data for healthy individuals, were available. ANALYSIS: Scores were compared using analysis of variance and multiple regression. RESULTS: One hundred and forty-six patients with CH-B, CH-C and PBC were included [mean age 47.1 years (+/-11.6), 41% female, 33% cirrhosis]. CH-C and PBC patients scored the lowest on all CLDQ, SF-36 and HUI domains compared with CH-B patients and healthy controls. CH-B patients had scores similar to the healthy population, measured by most CLDQ and SF-36 scales. However, the HUI scores for CH-B patients showed more impairment than population norms. Having CH-B and not having cirrhosis were predictive of utility and HRQL scores in multivariate models. CONCLUSIONS: CH-B patients have better HRQL than CH-C, PBC and population norms. CH-B patients' overall utility scores are lower than population norms.

The role of psychological factors in the fatigue of primary biliary cirrhosis.

Fatigue impairs the quality of life of primary biliary cirrhosis (PBC) patients. In this study, we explored the psychological factors and coping strategies in fatigued PBC patients. Patients participated in a semi-structured interview examining thoughts regarding the impact of fatigue and coping strategies. All completed the disease-specific quality-of-life tool, PBC-40, the Penn State Worry Questionnaire (PSWQ) (degree of habitual worry) and Hospital Anxiety and Depression Scale (HADS) (current anxiety and depression). PBC patients were allocated into high (>38, n=10) and low-fatigue (<38, n=14) groups. No differences were seen between high-fatigue and low-fatigue groups regarding age, marital status, employment status, PBC stage, years with diagnosis and years experiencing fatigue. High-fatigue participants were significantly more anxious (P=0.008), more depressed (P<0.001), and more likely to worry (<0.05). High-fatigue participants had more frequent thoughts about the impact of fatigue (P<0.005) and lower self-efficacy scores (P<0.001). In conclusion, PBC patients can experience profound distress associated with fatigue. PBC patients with high levels of fatigue seem to be more vulnerable to emotional distress, more likely to perceive that their quality of life has been negatively affected and are less confident to engage in everyday activities compared with those with low levels of fatigue.