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The British Society of Gastroenterology/UK-PBC primary biliary cholangitis treatment and management guidelines
  1. Gideon M Hirschfield1,2,3,
  2. Jessica K Dyson4,5,6,
  3. Graeme J M Alexander7,8,
  4. Michael H Chapman9,
  5. Jane Collier10,
  6. Stefan Hübscher3,11,
  7. Imran Patanwala12,13,
  8. Stephen P Pereira7,8,9,
  9. Collette Thain14,
  10. Douglas Thorburn7,8,
  11. Dina Tiniakos5,
  12. Martine Walmsley15,
  13. George Webster9,
  14. David E J Jones4,5,6
  1. 1 NIHR Birmingham Biomedical Research Centre, Birmingham, UK
  2. 2 University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK
  3. 3 Centre for Liver Research, Institute of Immunology and Immunotherapy, University of Birmingham, Birmingham, UK
  4. 4 Newcastle upon Tyne Hospitals NHS Foundation Trust, Newcastle upon Tyne, UK
  5. 5 Institute of Cellular Medicine, Newcastle University, Newcastle upon Tyne, UK
  6. 6 NIHR Newcastle Biomedical Research Centre, Newcastle, United Kingdom
  7. 7 Sheila Sherlock Liver Centre, Royal Free London NHS Foundation Trust, London, UK
  8. 8 UCL Institute for Liver and Digestive Health, Division of Medicine, University College London, London, UK
  9. 9 Department of Gastroenterology, University College London Hospitals NHS Foundation Trust, London, UK
  10. 10 Translational Gastroenterology Unit, Oxford University Hospitals, University of Oxford, Oxford, UK
  11. 11 Department of Cellular Pathology, University Hospitals Birmingham NHS Foundation Trust, Birmingham, UK
  12. 12 Department of Gastroenterology, Royal Liverpool and Broadgreen University Hospitals NHS Trust, Liverpool, UK
  13. 13 University of Liverpool, Liverpool, UK
  14. 14 PBC Foundation, Edinburgh, UK
  15. 15 PSC Support, Didcot, UK
  1. Correspondence to Professor Gideon M Hirschfield; g.hirschfield{at}bham.ac.uk

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 × 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.

  • autoimmune liver disease
  • care pathway
  • guidelines
  • ursodeoxycholic acid
  • obeticholic acid

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Footnotes

  • Contributors The development of these guidelines is as per the text.

  • Funding GMH is supported by the National Institute for Health Research (NIHR) Birmingham Biomedical Research Centre (BRC). GMH is supported by an EU Career development award. JKD is supported by the NIHR Rare Disease Translational Research Collaboration. GMH and DEJJ are investigators for UK-PBC, funded by the Medical Research Council, as a stratified medicine platform (www.uk-pbc.com).

  • Disclaimer This paper presents independent research funded and supported by the NIHR Birmingham Biomedical Research Centre at the University Hospitals Birmingham NHS Foundation Trust and the University of Birmingham. The views expressed are those of the author(s) and not necessarily those of the NHS, the NIHR or the Department of Health.

  • 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.

  • Patient consent Not required.

  • Provenance and peer review Not commissioned; externally peer reviewed.