The British Society of Gastroenterology/UK-PBC Primary Biliary Cholangitis Treatment and Management Guidelines

Gideon Hirschfield, Jessica K. Dyson, Graeme J Alexander, Michael Huw Chapman, Jane Collier, Stefan Hubscher, Imran Patanwala, Stephen P Pereira, Collette Thain, Douglas Thorburn, Dina Tiniakos, Martine Walmsley, George Webster, David E. Jones

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Abstract

Primary biliary cholangitis (formerly known as Primary Biliary Cirrhosis; PBC) is an autoimmune liver disease in which a cycle of immune mediated biliary epithelial cell injury, cholestasis and progressive fibrosis can culminate over time in an end-stage biliary cirrhosis. Both genetic and environmental influences are presumed relevant to disease initiation. PBC is most prevalent in women and those over the age of 50, but a spectrum of disease is recognised in adult patients globally; male sex, younger age at onset (<45) and advanced disease at presentation are baseline predictors of poorer outcome. As disease is increasingly diagnosed through the combination of cholestatic serum liver tests and presence of anti-mitochondrial antibodies, most presenting patients are not cirrhotic, and the term cholangitis is more accurate. Disease course is frequently accompanied by symptoms that can be burdensome for patients, and management of patients with PBC must address, in a life-long manner, both disease progression and symptom burden. Licenced therapies include Ursodeoxycholic acid (UDCA) and Obeticholic acid (OCA), alongside experimental new and re-purposed agents. Disease management focuses on initiation of UDCA for all patients, and risk stratification based on baseline and on-treatment factors, including in particular the response to treatment. Those intolerant of treatment with UDCA, or those with high risk disease as evidenced by UDCA treatment failure (frequently reflected in trial and clinical practice as an alkaline phosphatase (ALP) >1.67 x upper limit of normal (ULN) and/or elevated bilirubin) should be considered for second-line therapy, of which OCA is the only currently licenced National Institute For Health and Clinical Excellence (NICE) recommended agent. Follow up of patients is life-long and must address not just treatment of disease but management of associated symptoms.
Original languageEnglish
JournalGut
Early online date28 Mar 2018
DOIs
Publication statusE-pub ahead of print - 28 Mar 2018

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