RT Journal Article
SR Electronic
T1 The British Society of Gastroenterology/UK-PBC primary biliary cholangitis treatment and management guidelines
JF Gut
JO Gut
FD BMJ Publishing Group Ltd and British Society of Gastroenterology
SP 1568
OP 1594
DO 10.1136/gutjnl-2017-315259
VO 67
IS 9
A1 Gideon M Hirschfield
A1 Jessica K Dyson
A1 Graeme J M Alexander
A1 Michael H Chapman
A1 Jane Collier
A1 Stefan Hübscher
A1 Imran Patanwala
A1 Stephen P Pereira
A1 Collette Thain
A1 Douglas Thorburn
A1 Dina Tiniakos
A1 Martine Walmsley
A1 George Webster
A1 David E J Jones
YR 2018
UL http://gut.bmj.com/content/67/9/1568.abstract
AB 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 the disease is increasingly diagnosed through the combination of cholestatic serum liver tests and the presence of antimitochondrial 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. Licensed 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 >1.67 × upper limit of normal and/or elevated bilirubin) should be considered for second-line therapy, of which OCA is the only currently licensed National Institute for Health and Care Excellence recommended agent. Follow-up of patients is life-long and must address treatment of the disease and management of associated symptoms.