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,4 and the UK-PBC Consortium 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;58:273-283)

Age at presentation is a major risk factor for progression to transplant (as well as UDCA non-response) in PBC. Although both confirmatory longitudinal studies, and studies utilising objective as well as subjective measures of function, are needed if we are to address the question definitively, we found no evidence of improved systemic symptoms after liver transplantation in PBC and patients should be advised accordingly. Consideration needs to be given to enhancing rehabilitation approaches to improve function and life quality after liver transplant for PBC.

BSG abstracts Introduction PBC is characterised by loss of small intrahepatic bile ducts, and in a significant proportion of patients by persistent fatigue. Genes regulating inflammatory pathways have been strongly associated with PBC in population-scale genetic studies, implicating inflammation in disease pathogenesis. Animal models of cholestasis, a biological process in PBC, have demonstrated fatigue-like behaviour appearing to result from responses to inflammatory cytokine release in the brain. Elevation of inflammatory cytokines has therefore, unsurprisingly, been postulated as an underlying mechanism for fatigue in PBC as well as other chronic inflammatory conditions. However, more recently data demonstrating that fatigue is not proportional to liver disease severity in PBC has questioned this presumed correlation between inflammation and fatigue. This study aimed to explore the serum cytokine profile in PBC compared to healthy controls, and to correlate this picture of inflammation status with fatigue severity. Methods 68 patients from the Newcastle sector of the UK-PBC cohort and 9 healthy controls provided a morning peripheral blood sample and completed the PBC-40, a validated disease-specific quality of life measure with a fatigue domain. Sera were derived using standard protocols and stored at-80°C prior to multiplex cytokine quantification using the MSD platform. Results PBC patients showed significant elevation of IFN-γ (median 2.4pg/ml[IQR 1.

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