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:-) Primary biliary cirrhosis (PBC), the commonest autoimmune liver disease, affects the lives of patients in numerous ways, including through progression to cirrhosis and endstage liver disease with complications of liver failure, portal hypertension, and hepatocellular carcinoma. Symptoms, which include http://www.selleckchem.com/products/Everolimus(RAD001).html the archetypal cholestatic pruritus, are reported by many patients. Recent advances in primary therapy with ursodeoxycholic acid (UDCA) and transplantation

PXD101 have reduced the risk to life from PBC substantially,4, 5 and approaches to primary and second-line disease processes are outlined in treatment guidelines.6, 7 Patient reports suggest, however, that the clinical impact of PBC is more complex than that of a slowly progressive chronic liver disease complicated in some by treatable pruritus.8 There is significant impairment of quality of life (QOL) in a substantial proportion.9, 10 The factors underpinning this QOL impairment appear to be multiple and complex, but include fatigue, cognitive symptoms, symptoms of social and emotional dysfunction, sleep disturbance, and depression.11 There is also significant debate as to whether these symptoms are a manifestation of the disease process itself, associated processes, or a reflection of the age, gender, and comorbidity of the selected PBC patient populations studied.12, 13 The uncertainty about the nature of the complex symptoms of PBC

is compounded by the fact that studies addressing these issues have often been performed in small, selected study populations and in centers with a particular interest in such symptoms. The aim of the current study was to use the unique resource of the UK-PBC Patient Cohort, the largest in the world, selleck chemicals llc which has recruited participants from every clinical center in the UK to address the issue of QOL impairment in PBC and those factors underpinning it. A cross-sectional cohort study of patients recruited to the UK-PBC Patient Cohort, which was established initially to undertake a UK PBC genome-wide association study (GWAS).14, 15 Patients were phenotyped with regard to symptoms and QOL using established and validated measures.16 In order to compare the symptom values for PBC-related symptoms quantified using the PBC-40, a version of this measure suitable for completion by control subjects was developed and validated as part of the study protocol.