The British Society of Gastroenterology/UK-PBC primary biliary cholangitis treatment and management guidelines.

{"article_title":"The British Society of Gastroenterology\/UK-PBC primary biliary cholangitis treatment and management guidelines.","author":"Hirschfield GM, Dyson JK, Alexander GJM, Chapman MH, Collier J, H\u00fcbscher S, Patanwala I, Pereira SP, Thain C, Thorburn D, Tiniakos D, Walmsley M, Webster G, Jones DEJ","journal_title":"Gut","issn":"1468-3288","isbn":"","publication_date":"2018-09-01","volume":"67","issue":"9","first_page":"1568","page_count":"","accession_number":"29593060","doi":"10.1136\/gutjnl-2017-315259","publisher":"British Medical Assn","doctype":"Guideline","subjects":"Chenodeoxycholic Acid analogs & derivatives; Cholagogues and Choleretics therapeutic use; Cholangitis diagnosis; Cholangitis therapy; Gastroenterology; Ursodeoxycholic Acid therapeutic use; Alanine Transaminase blood; Alkaline Phosphatase blood; Autoantibodies blood; Bilirubin blood; Biomarkers blood; Chenodeoxycholic Acid therapeutic use; Cholangitis blood; Disease Progression; Humans; Liver Cirrhosis, Biliary; Mitochondria immunology; Predictive Value of Tests; Risk Assessment; Risk Factors; Sensitivity and Specificity; Societies, Medical; Treatment Outcome; United Kingdom","interest_area":["Gastroenterology"],"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 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 \u00d7 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. Competing Interests: Competing interests: GMH: Advisory boards for Falk, GfK, GSK, Intercept, Novartis; consultancy for CymaBay; clinical trial investigator for Falk, FF Pharma, Gilead, GSK, Intercept, Novartis, NGM Bio, Shire. JKD: member of BSG and BASL. GJMA: department receives\/received financial support for clinical trials from GSK, Intercept and Cymabay. IP: honorarium received for chairing sessions\/lectures\/meetings and sponsorship to attend Falk Symposium from Dr Falk Pharma. DT: department receives\/received financial support for hosting and speaking at meetings from Dr Falk Pharma. MW: holds a voluntary position as the chair of Trustees for PSC Support. DEJJ: grant funding from Pfizer and Intercept; consultancy for Intercept, GSK and Novartis; speaker bureau for Dr Falk Pharma. Member of medical advisory board for PBC Foundation. \u00a9 Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.","url":"https:\/\/search.ebscohost.com\/login.aspx?direct=true&db=mdl&AN=29593060","isPdfLink":false,"isSAML":false,"an":"29593060","number_other":"","type_pub":"","issn_electronic":"1468-3288","languages":"English","language":"eng","date_entry":"Date Created: 20180330 Date Completed: 20180917 Latest Revision: 20230928","date_update":"20240105","titleSource":"Gut [Gut] 2018 Sep; Vol. 67 (9), pp. 1568-1594. Date of Electronic Publication: 2018 Mar 28.","date_pub_cy":"","type_document":"","contract_publisher":"","authored_on":"2018-09-01","description":"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 (&lt;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 &gt;1.67\u2009&#215; 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.&lt;br \/&gt;Competing Interests: Competing interests: GMH: Advisory boards for Falk, GfK, GSK, Intercept, Novartis; consultancy for CymaBay; clinical trial investigator for Falk, FF Pharma, Gilead, GSK, Intercept, Novartis, NGM Bio, Shire. JKD: member of BSG and BASL. GJMA: department receives\/received financial support for clinical trials from GSK, Intercept and Cymabay. IP: honorarium received for chairing sessions\/lectures\/meetings and sponsorship to attend Falk Symposium from Dr Falk Pharma. DT: department receives\/received financial support for hosting and speaking at meetings from Dr Falk Pharma. MW: holds a voluntary position as the chair of Trustees for PSC Support. DEJJ: grant funding from Pfizer and Intercept; consultancy for Intercept, GSK and Novartis; speaker bureau for Dr Falk Pharma. Member of medical advisory board for PBC Foundation.&lt;br \/&gt; (&#169; Article author(s) (or their employer(s) unless otherwise stated in the text of the article) 2018. All rights reserved. No commercial use is permitted unless otherwise expressly granted.)","upload_link":"https:\/\/search.ebscohost.com\/login.aspx?direct=true&site=eds-live&scope=site&db=mdl&AN=29593060&authtype=shib&custid=ns346513&group=main&profile=eds","no_of_pages":"","authored_by":"Hirschfield GM, Dyson JK, Alexander GJM, Chapman MH, Collier J, H\u00fcbscher S, Patanwala I, Pereira SP, Thain C, Thorburn D, Tiniakos D, Walmsley M, Webster G, Jones DEJ"}