Journal
JOURNAL OF HEPATOLOGY
Volume 67, Issue 1, Pages 145-172Publisher
ELSEVIER
DOI: 10.1016/j.jhep.2017.03.022
Keywords
Cholestasis; Guidelines; Care pathway; Liver
Categories
Funding
- European Association for the Study of the Liver
- DFG
- Helmut and Hannelore Greve Foundation
- YAEL-Foundation
- National Institute for Health Research (NIHR) Birmingham Liver Biomedical Research Centre
- Newcastle Biomedical Research Centre
- Medical Research Council
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Primary biliary cholangitis (PBC) is a chronic inflammatory autoimmune cholestatic liver disease, which when untreated will culminate in end-stage biliary cirrhosis. Diagnosis is usually based on the presence of serum liver tests indicative of a cholestatic hepatitis in association with circulating antimitochondrial antibodies. Patient presentation and course can be diverse and risk stratification is important to ensure all patients receive a personalised approach to their care. The goals of treatment and management are the prevention of end-stage liver disease, and the amelioration of associated symptoms. Pharmacologic approaches in practice, to reduce the impact of the progressive nature of disease, currently include licensed therapies (ursodeoxycholic acid and obeticholic acid) and off-label therapies (fibric acid derivatives, budesonide). These clinical practice guidelines summarise the evidence for the importance of a structured, life-long and individualised, approach to the care of patients with PBC, providing a framework to help clinicians diagnose and effectively manage patients. (C) 2017 European Association for the Study of the Liver. Published by Elsevier B.V. All rights reserved.
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