PBC-40 Questionnaire Research Articles

Total and individual PBC-40 scores are reliable for the assessment of health-related quality of life in Greek patients with primary biliary cholangitis.

Primary biliary cholangitis (PBC) has been long associated with impairment of various aspects of health-related quality of life (HRQoL) with substantial differences among populations. This study evaluated for the first-time the HRQoL in Greek PBC patients in conjunction with clinical and laboratory parameters of patients. We analyzed prospectively collected data regarding the HRQoL by using the PBC-40 and SF-36 questionnaires in 374 Greek PBC patients and 131 age- and sex-matched non-PBC controls. The PBC-40 questionnaire is a reliable tool for HRQoL assessment in Greek PBC patients (Cronbach's α > 0.7 for all domains). Implementation of PBC-40 and SF-36 demonstrated significant impairment of HRQoL in Greek PBC patients compared to controls (P < 0.001 for all comparisons). Emotional dysfunction, social impairment, and fatigue (100%, 80.5% and 78%, respectively) were amongst those with the highest, while cognitive dysfunction (32%) with the least impact on quality of life. Fatigue was associated with female sex (P = 0.02), longer disease duration (P = 0.01), presence of cirrhosis (P = 0.02) and positivity for PBC-specific ANA (P < 0.05), while social dysfunction with increased age (P < 0.001), longer disease duration (P < 0.001) and presence of cirrhosis (P = 0.004). Living in urban areas was linked to impaired social function (P = 0.04), cognition (P = 0.02), fatigue (P = 0.04) and increased total PBC-40 score (P = 0.01). Implementation of PBC-40 and SF-36 revealed impaired HRQoL in Greek PBC patients with fatigue, social and emotional dysfunction exerting the highest impact. However, total, and individual PBC-40 scores were lower than that reported in studies from Northern/Central Europe and Canada. Deranged HRQoL was associated with severity of liver disease and presence of PBC-specific ANA.

Health related quality of life in patients with primary biliary cholangitis

Aim: To assess the health related quality of life (HRQOL) in patients diagnosed with primary biliary cholangitis (PBC). Material and methods: A single-centered, prospective, cross-sectional study was conducted among patients diagnosed with primary biliary cholangitis in the Gastroenterology out-patient clinic at Gazi University Hospital, Ankara. Data was collected using a sociodemographic information form, hospital medical database and a PBC-40 questionnaire. Statistical comparison analysis was performed using Chi-square test for categorical variables and Mann-Whitney U test for continuous variables. P values less than 0.05 were considered significant. Results: A total of 37 PBC patients were included in the study. The mean age of the participants was 54.2±8.6. When evaluating the PBC-40 questionnaire scores from each domain in terms of severity, we found that majority of patients in itch %40.4 (n=19) and social %42,6 (n=20) domains had mild severity, as for fatigue %42.6 (n=20), cognitive %36 (n=17), emotional %40.4 (n=19) and other symptoms %44.7 ((n=21) domains had moderate severity. A statistically significant difference was found between AMA positive and AMA negative patient groups in the itch domain (p=0.007). Discussion: With this study on a Turkish based population using the PBK-40 questionnaire we were able to demonstrate that patients with PBC had a significantly impaired HRQOL and fatigue was the most seen symptom.

Ethnicity, disease severity, and survival in Canadian patients with primary biliary cholangitis.

We investigated associations between ethnicity, survival, and disease severity in a diverse Canadian cohort of patients with primary biliary cholangitis (PBC). Patients with PBC were included from the Canadian Network for Autoimmune Liver Disease. Ethnicity was defined using a modified list adopted from Statistics Canada, and ethnicities with small samples were grouped. Clinical events were defined as liver decompensation, HCC, liver transplantation, or death. Clinical event-free and liver transplantation-free survival were analyzed using Cox regression. Trajectories of serum liver function tests were assessed over time using mixed-effects regression. Health-related quality of life was assessed using the Short Form 36, the PBC-40 questionnaire, and the 5-D Itch scale and analyzed using mixed-effects regression. The cohort included 1538 patients with PBC from six sites and was comprised of 82% White, 4.7% Indigenous, 5.5% East Asian, 2.6% South Asian, and 5.1% miscellaneous ethnicities. Indigenous patients were the only ethnic group with impaired liver transplant-free and event-free survival compared to White patients (HR, 3.66; 95% CI, 2.23-6.01; HR, 3.09; 95% CI, 1.94-4.92). Indigenous patients were more likely to have a clinical event before diagnosis (10%) than all other ethnic groups despite similar age at diagnosis. Indigenous patients presented with higher alkaline phosphatase, total bilirubin, and GLOBE scores than White patients; and these relative elevations persisted during follow-up. Indigenous Canadians with PBC present with advanced disease and have worse long-term outcomes compared to White patients.

Seladelpar improved measures of pruritus, sleep, and fatigue and decreased serum bile acids in patients with primary biliary cholangitis.

Primary biliary cholangitis (PBC) can result in life-altering cholestatic pruritus and fatigue, but treatment options are limited. Seladelpar, a peroxisome proliferator-activated receptor-delta (PPARδ) agonist, has demonstrated potent anti-cholestatic effects in clinical studies. This open-label, uncontrolled phase 2 study in PBC patients evaluated the effects of 1-year of seladelpar treatment on measures of pruritus and quality of life. Self-reported experiences of 101 PBC patients were collected at baseline and after 1year of seladelpar treatment using the pruritus visual analog scale (VAS), 5D-itch scale, and PBC-40 questionnaires along with bile acid profiles. In patients with moderate-to-severe pruritus, substantial improvement in pruritus was seen in 58% and 93% of patients in 5/10mg and 10mg treatment groups, respectively. After 1year, patients reporting improvement substantially outnumbered those who worsened in the total 5-D itch (including individual domains) and PBC-40 (itch and fatigue domains) questionnaires. Improvement in sleep disturbance at 1-year was reported in 81% (5/10mg) and 78% (10mg) of the patients with baseline itch-related sleep disturbance by 5-D itch score with similar results using the PBC-40 sleep questionnaire. Seladelpar-treated patients had significant reductions of 46% (5/10mg) and 31% (10mg) in the serum bile acid precursor C4 and reductions of up to 38% in serum bile acids. Seladelpar treatment for 1year led to consistent improvement in both symptom burden and biochemical response, suggesting its potential as a single agent to address two key unmet needs in PBC patients.

Evaluation of the effects of additional therapy with Berberis vulgaris oxymel in patients with refractory primary sclerosing cholangitis and primary biliary cholangitis: A quasi-experimental study.

There are several studies reporting the therapeutic effects of Berberis vulgaris on liver diseases. This study was done with the purpose of examining the effect of B. vulgaris oxymel (BO) in patients with refractory primary sclerosing cholangitis (PSC) and primary biliary cholangitis (PBC), who did not respond to current treatment. Patients with PSC or PBC who were receiving ursodeoxycholic acid (UDCA, 13-15 mg/kg/day) for at least six months, but their serum levels of alkaline phosphatase (ALP) were still 1.5 folds higher than the normal upper limit during the last six months, were asked to participate in this quasi-experimental study. Patients were asked to take 0.5 ml/kg/day of BO two times a day for three months along with UDCA. At the end of the study, serum levels of ALP, aspartate aminotransferase (AST), alanine aminotransferase (ALT), gamma-glutamyl transferase (GGT), total bilirubin (TB), direct bilirubin (DB), and creatinine as well as prothrombin time (PT), international normalized ratio (INR) and quality of life (QOL) based on PBC-40 questionnaire were assessed as outcomes. Our results showed that BO notably attenuated the serum levels of ALP, AST, ALT, GGT, TB, and DB, as well as PT and INR and significantly improved QOL. For first time, we showed that additional therapy with BO has a promising effect in the treatment of refractory PSC and PBC.

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.

Symptom Burden and Treatment Response in Patients with Primary Biliary Cholangitis (PBC).

Primary biliary cholangitis (PBC) is a chronic, cholestatic liver disease that can lead to end-stage liver disease and impairs the quality of life. At current, most data come from few large cohorts. This cross-sectional study evaluated treatment response and symptom burden in patients with PBC in Germany to expand the available data. A total of 140 PBC patients were prospectively enrolled at the outpatient liver clinic of the University Medical Center in Mainz starting in June 2016. Historic and current response rates of UDCA treatment were determined using published binary models. Symptom burden was assessed using the PBC-40 questionnaire. The primary treatment response ranged between 73 and 86% depending on the definition used. Importantly, this response rate was maintained over a median time of 5years in follow-up. The highest symptom burden was observed for fatigue and emotional (2.4 ± 1; 2.3 ± 1.1 of 5), while pruritus (1.1 ± 1.1 of 5) had the lowest scores. IgG correlated with the PBC-40 domain social (r = 0.211, p = 0.032), while HDL inversely correlated with the symptom burden of pruritus (r = - 0.236; p = 0.018). In this tertiary care cohort, 75% of the patients showed biochemical response after 1year according to the acknowledged Paris II criteria. Patients reported a significant symptom burden, and the domain fatigueof the PBC-40 was most prominently impaired.

PBC-10: a short quality of life measure for clinical screening in primary biliary cholangitis.

Current guidelines in primary biliary cholangitis ( PBC) recommend routine screening for symptoms. However, at present there are no validated practical tools suitable for screening use in practice. To develop a short quality of life questionnaire for PBC METHODS: The short PBC HRQL questionnaire was derived and validated by analysing the PBC-40 questionnaires from the UK-PBC Research Cohort. Construct validity was assessed using the European Quality of Life Five Dimensions (EQ5D) questionnaire. Test-retest analysis was done by asking a subgroup of patients to complete the questionnaire twice within 2-4weeks. A total of 2219 patients completed PBC-40 questionnaire in 2013. Stepwise regression identified 10 questions that contributed to more than 95% of the PBC-40 score variance and covered the main domains of PBC. The short HRQL questionnaire, PBC-10, had good internal consistency (Cronbach's α 0.905) and item-total correlations. PBC-10 demonstrated no ceiling effects but a floor effect was noted. Further validation on 2502 patients who completed the PBC questionnaire in 2017 confirmed the psychometric properties of PBC-10. Further analysis on 186 patients showed that PBC-10 demonstrated good internal consistency (Cronbach's α=0.936), had good reproducibility (intra-class correlation coefficient=0.945), good correlation with the EQ5D (r=.736), and was responsive to change. A change of 4 points in the PBC-10 score would be considered clinically important. PBC-10 is a short and valid questionnaire for assessing the HRQL in patients with PBC in clinical practice.

Effects of liver transplantation on health-related quality of life in patients with primary biliary cholangitis.

Patients with primary biliary cholangitis (PBC) have decreased health-related quality of life (HRQoL). Here, we investigate HRQoL in two cohorts of transplanted patients with PBC and compare their results to healthy subjects. We used generic SF-36 and disease-specific PBC-40 questionnaires to evaluate HRQoL in 26 patients with PBC (23 females, age 59.4±5.7years) before and after liver transplantation (LT), and in 107 patients with PBC (99 females, age 62.8±6.7years) who were previously transplanted. The control group was comprised of 60 healthy controls (55 females, age 54.6±8.8years). Health-related quality of life improved after LT in 85% of PBC patients. The SF-36 measure showed significant (all P<0.05) improvements in the majority of domains after LT, and in the summary scores both physical and mental. We also documented significant improvements in pruritus andfatigue after LT (all P<0.01). However, liver graft recipients had significantly worse physical functioning, physical role, and emotional role domains, and physical component score (all P<0.001), as compared to healthy subjects. No differences in HRQoL were detected between patients evaluated after short and prolonged post-LT periods (P>0.05). Liver transplantation substantially improves most aspects of life quality in PBC patients. Nevertheless, their HRQoL remains worse in comparison to healthy individuals, mainly in physical aspects.

Effect of S-adenosyl-L-methionine on liver biochemistry and quality of life in patients with primary biliary cholangitis treated with ursodeoxycholic acid. A prospective, open label pilot study.

Chronic liver disease induces an acquired deficiency of S-adenosyl-L-methionine (SAMe) leading to impairment of detoxifying processes in the liver. Ursodeoxycholic acid (UDCA) represents the standard treatment in primary biliary cholangitis (PBC). As both compounds exert their hepatoprotective effects by different mechanisms, it is conceivable that when used together their effect might be additive. The aim of this study was to analyse the effect of SAMe supplementation on liver biochemistry and health-related quality of life (HRQoL) in patients with PBC, treated with UDCA. In this prospective pilot, proof of the principle, non-randomized and open label study we enrolled 24 patients with PBC treated with UDCA for at least 6 months. They had received both UDCA in a standard dose of 13-15 mg/kg b.w. and SAMe in the dose of 1200 mg daily over a period of 6 months. A group of 24 patients with PBC treated with UDCA served as control for liver biochemistry (Study registered on the platform ClinicalTrials.gov under ID: NCT02557360). We observed a significant decrease of ALP, GGT and total cholesterol in non-cirrhotic patients treated with SAMe. There was also a significant improvement of fatigue and pruritus in PBC-40 questionnaire and amelioration of anxiety in STAI 2 questionnaire in the SAMe group. Treatment with SAMe neither increased sulfation capacity of the liver nor had an effect on fibroblast growth factor-19 serum levels. Our pilot study demonstrates a positive effect of adding SAMe to UDCA in non-cirrhotic patients with PBC.

Pilot study with IBAT inhibitor A4250 for the treatment of cholestatic pruritus in primary biliary cholangitis

Pruritus is a common complication of cholestatic liver diseases. Inhibition of the ileal bile acid transporter (IBAT/ASBT) may emerge as treatment option. Our aim was to assess tolerability and effect on pruritus of the selective IBAT inhibitor A4250 in patients with primary biliary cholangitis (PBC). Ten patients with PBC and bile acid sequestrant treatment of cholestatic pruritus were after a two-week wash out of the bile acid sequestrant treated with either 0.75 mg (n = 4) or 1.5 mg (n = 5) of A4250 for four weeks. Patients’ pruritus was assessed by Visual Analogue Scale (VAS), 5-D itch scale and the pruritus module of the PBC40 questionnaire. Plasma bile acids and 7α-hydroxy-4-cholesten-3-one were measured by UPLC-MS/MS, plasma fibroblast growth factor 19 by ELISA, and serum autotaxin activity by homemade assay. All nine patients exposed to A4250 reported a remarkable improvement in pruritus, until none or mild according to 5-D itch, VAS and PBC40 pruritus. Five patients finished the study prematurely due to abdominal pain (5/5) and diarrhoea (4/5). The high incidence of probably bile acid malabsorption-related diarrhoea and abdominal pain in the bile acid sequestrant pre-treated population indicates that the start dose of A4250 may have been too high for adult patients.

Rituximab for the treatment of fatigue in primary biliary cholangitis (formerly primary biliary cirrhosis): a randomised controlled trial

Background Primary biliary cirrhosis (PBC) is an autoimmune liver disease, and 50% of patients with this disease experience fatigue. This is a debilitating symptom affecting quality of life and resulting in social isolation, which is highlighted by patients as a research priority. PBC is characterised immunologically by the presence of high-titre autoantibodies that are directed at the pyruvate dehydrogenase complex (PDC) and are highly effective at blocking its energy generation function. We hypothesised that if anti-PDC antibodies were a driver of fatigue through bioenergetic dysfunction, then the B-cell-targeting biological agent rituximab (MabThera®, Roche Products Ltd, Welwyn Garden City, UK) might be a therapeutic option. Objective To assess whether or not rituximab safely improved moderate or severe fatigue in PBC patients. Design A Phase II, double-blind, randomised controlled trial comparing rituximab with placebo in fatigued PBC patients. Randomisation was conducted using a web-based system. Participants received two infusions on days 1 and 15 and were followed up at 3, 6, 9 and 12 months. Setting A single-centre UK study in Newcastle upon Tyne Hospitals NHS Foundation Trust. Participants Seventy-one participants aged ≥ 18 years with PBC and moderate or severe fatigue (score of &gt; 33 on the PBC-40 fatigue domain) were screened. The PBC-40 questionnaire is a fully validated disease-specific health-related quality-of-life measure for use in patients with PBC. Fatigue, with a maximum score of 55, is one of its six domains. Fifty-seven participants were randomised to the trial, 55 of whom reached the primary end-point assessment. Intervention Participants were randomised in a 1 : 1 ratio to receive either rituximab (1000 mg) or a saline intravenous infusion (placebo) on days 1 and 15. The infusions were delivered in a double-blind manner using the same protocol. Main outcome measures The primary outcome measure was the PBC-40 fatigue domain at 3 months, assessed on an intention-to-treat basis. Secondary outcome measures included markers of bioenergetics function (anaerobic threshold and post-exercise muscle pH assessed using magnetic resonance imaging) and physical activity levels. Impact on biochemical markers of liver disease severity was assessed as an experimental outcome. Results Rituximab therapy was safe, with no serious adverse events linked to the drug. There was no statistically significant difference in fatigue score at 3 months between the rituximab and placebo arms [adjusted mean difference –0.9, 95% confidence interval (CI) –4.6 to 3.1]. However, improvement in fatigue was observed in both arms {mean score decreasing from 41.2 [standard deviation (SD) 5.5] to 36.2 (SD 8.4) in the rituximab arm and from 43.0 (SD 5.9) to 38.1 (SD 8.7) in the placebo arm}. There was little difference in any of the secondary outcomes between arms. However, anaerobic threshold improved significantly in the rituximab arm (adjusted mean difference at 3 months 1.41, 95% CI 0.03 to 2.80). No change in muscle bioenergetics characteristics was seen. A suggestive improvement in liver biochemistry was observed. Limitations Recruitment was lower than the original target, leading to a reduction in study power. A clinically significant placebo effect on PBC-40 fatigue scores was seen. Conclusions Rituximab is ineffective for the treatment of fatigue in unselected PBC patients despite metabolic modulation through improvement of anaerobic threshold. Future work Results from the trial demonstrate that metabolic effect of rituximab is not translated into clinical benefit. This will help to guide us to design future trials and when looking at completely different targets. Trial registration Current Controlled Trials ISRCTN03978701, ClinicalTrials.gov identifier NCT02376335 and EudraCT number 2012-000145-12. Funding This project was funded by the National Institute for Health Research (NIHR) Efficacy and Mechanism Evaluation programme and will be published in full in Efficacy and Mechanism Evaluation; Vol. 5, No. 2. See the NIHR Journals Library website for further project information. Additional funding was received from the Medical Research Council and a Department of Health and Social Care subvention.

Prospective evaluation of ursodeoxycholic acid withdrawal in patients with primary sclerosing cholangitis

Ursodeoxycholic acid (UDCA) is no longer recommended for management of adult patients with primary sclerosing cholangitis (PSC). We undertook a prospective evaluation of UDCA withdrawal in a group of consecutive patients with PSC. Twenty six patients, all treated with UDCA (dose range: 10-15 mg/kg/day) were included. Paired blood samples for liver biochemistry, bile acids, and fibroblast growth factor 19 (FGF19) were collected before UDCA withdrawal and 3 months later. Liquid chromatography/tandem mass spectrometry was used for quantification of 29 plasma bile acid metabolites. Pruritus and health-related quality of life (HRQoL) were assessed with a 10-point numeric rating scale, the Medical Outcomes Study Short Form-36 (SF-36), and PBC-40 questionnaires. UDCA withdrawal resulted in a significant deterioration in liver biochemistry (increase of alkaline phosphatase of 75.6%; P<0.0001; gamma-glutamyl transpeptidase of 117.9%, P<0.0001; bilirubin of 50.0%, P<0.001; alanine aminotransferase of 63.9%, P<0.005; and aspartate aminotransferase of 45.0%, P<0.005) and increase of Mayo Risk Score for PSC (change from baseline of +0.5 point; P<0.003). Bile acid analysis revealed a significant decrease in lithocholic acid and its derivatives after UDCA withdrawal, but no effect on concentrations of primary bile acids aside from an increased accumulation of their taurine conjugates. After UDCA removal cholestatic parameters, taurine species of cholic acid and chenodeoxycholic acid correlated with serum FGF19 levels. No significant effect on HRQoL after UDCA withdrawal was observed; however, 42% of patients reported a deterioration in their pruritus. At 3 months, discontinuation of UDCA in patients with PSC causes significant deterioration in liver biochemistry and influences concentrations of bile acid metabolites. A proportion of patients report increased pruritus, but other short-term markers of quality of life are unaffected.

Association between cytotoxic T‐lymphocyte antigen 4 gene polymorphisms and primary biliary cirrhosis in Chinese population: data from a multicenter study

The cytotoxic T-lymphocyte antigen 4 (CTLA4) gene polymorphisms have been shown to be associated with the risk of primary biliary cirrhosis (PBC). The study aimed to confirm the associations of CTLA4 gene polymorphisms with risk of PBC and patients' quality of life in Chinese population. A total of 312 female PBC patients from Chinese Han population were included as case, and 375 age-matched female healthy volunteers were included as control. Four single nucleotide polymorphisms (SNPs) including rs231775, rs3087243, rs231725, and rs5742909 were genotyped. The differences of genotype and allele distributions between PBC patients and healthy controls were assessed. The relationship between CTLA4 gene polymorphisms and healthy status of PBC patients were then investigated through comparisons of the domain scores of PBC-40 questionnaire between different genotype categories of each single nucleotide polymorphism. The frequencies of G allele at rs231775 and A allele at rs231725 were both significantly increased in PBC patients when compared with normal controls (P < 0.001, odds ratio = 1.44, 95% confidence interval = 1.24-1.67 for rs231775; P < 0.001, odds ratio = 1.29, 95% confidence interval = 1.12-1.48 for rs231725). Besides, patients carrying A allele of rs3087243 had significantly lower score of fatigue domain than those carrying G allele (2.5 ± 0.8 vs 3.9 ± 1.3, P < 0.001). This study revealed that CTLA4 gene polymorphism might be associated with susceptibility of PBC. G allele of rs231775 and A allele of rs231725 were significantly associated with the risk of PBC. In addition, patients carrying A allele of rs3087243 could have significantly better quality of life than those carrying G allele.

The effect of liver transplantation on fatigue in patients with primary biliary cirrhosis: A prospective study

The role of liver transplantation (LT) for the relief of fatigue in patients with primary biliary cirrhosis (PBC) is unclear, and while many centers exclude fatigue as an indication for transplantation, there have been no studies to prospectively evaluate the impact of LT on fatigue. We aimed at assessing the severity of fatigue in LT candidates with PBC and the impact of LT on fatigue. In a prospective, longitudinal study, we used the PBC-40 questionnaire in 49 adult patients with PBC at listing and at 6, 12, and 24 months after LT and in two sex- and age-matched cohorts of community controls and non-transplanted PBC patients. Correlation analysis was used to assess the relationship between liver function and fatigue. ANOVA was used to compare the variation of fatigue score before and after LT. There was no correlation between MELD and fatigue before LT (r(2)=0.01). Overall, the fatigue score after LT was substantially lower than before LT, falling from 40.7 ± 11.4 pre-transplant to 27.7 ± 9.5, 28.7 ± 10.1, 26.2 ± 10.1 (p<0.0001) at 6, 12, and 24 months after LT, respectively. The same improvement of fatigue was observed in both low-MELD (<17) and high-MELD (≥ 17) patients. Improvement in fatigue was also evident in the comparison with a "non-transplant PBC" control group (31.1 ± 11.6, p=0.03). However, 44% of the total cohort, and 47% of those with low-MELD, for whom the probability of dying of LT may be higher than that of dying without LT, had moderate to severe fatigue (defined as a fatigue score ≥ 29) at two years after LT. Moreover, fatigue scores at two years were higher in the transplant PBC cohort compared to a cohort of community controls (17.8 ± 5.9, p<0.0001). Liver transplantation is associated with improvement in fatigue in patients with PBC. However, a substantial proportion of patients continue to suffer from significant fatigue after two years. Whether the improvement is enough to justify organ allocation in patients with fatigue alone, without liver failure, is still an open issue. Certainly, in the era of organ shortage, with many patients dying waiting for a graft, this may not represent the optimal use of donated deceased organs.

S1782 Health Related Quality of Life in Patients with Primary Biliary Cirrhosis Using a Japanese Version of the PBC-40 Questionnaire

S1782 Health Related Quality of Life in Patients with Primary Biliary Cirrhosis Using a Japanese Version of the PBC-40 Questionnaire