Critical shortfalls in the management of PBC: Results of a UK-wide, population-based evaluation of care delivery

Nadir Abbas; Rachel Smith; Steven Flack; Vikram Bains; Richard J Aspinall; Rebecca L Jones; Laura Burke; Douglas Thorburn; Michael Heneghan; Andrew Yeoman; Joanna Leithead; Conor Braniff; Andrew Robertson; Chris Mitchell; Collette Thain; Robert Mitchell-Thain; David Jones; Palak J Trivedi; George F Mells; Laith Alrubaiy; UK-PBC audit group

doi:10.1016/j.jhepr.2023.100931

Critical shortfalls in the management of PBC: Results of a UK-wide, population-based evaluation of care delivery

JHEP Rep. 2023 Oct 16;6(1):100931. doi: 10.1016/j.jhepr.2023.100931. eCollection 2024 Jan.

Authors

Nadir Abbas^{1

2

3}, Rachel Smith^{4

5}, Steven Flack⁴, Vikram Bains⁵, Richard J Aspinall⁶, Rebecca L Jones⁷, Laura Burke⁷, Douglas Thorburn⁸, Michael Heneghan⁹, Andrew Yeoman¹⁰, Joanna Leithead¹¹, Conor Braniff¹², Andrew Robertson¹³, Chris Mitchell¹⁴, Collette Thain¹⁴, Robert Mitchell-Thain¹⁵, David Jones^{16

17}, Palak J Trivedi^{1

2

3

18}, George F Mells^{4

5}, Laith Alrubaiy^{19

20}; UK-PBC audit group

Affiliations

¹ National Institute for Health and Care Research (NIHR) Birmingham Biomedical Research Centre (BRC), Centre for Liver and Gastrointestinal Research, University of Birmingham, Birmingham, UK.
² Liver Unit, University Hospitals Birmingham Queen Elizabeth, Birmingham, UK.
³ Institute of Immunology and Immunotherapy, University of Birmingham, Birmingham, UK.
⁴ Academic Department of Medical Genetics, University of Cambridge, Cambridge, UK.
⁵ Cambridge Liver Unit, Cambridge University Hospitals NHS Foundation Trust, Cambridge, UK.
⁶ Department of Gastroenterology and Hepatology, Portsmouth Hospitals University NHS Trust, Queen Alexandra Hospital, Portsmouth, UK.
⁷ Department of Hepatology, Leeds Teaching Hospitals NHS Trust, Leeds, UK.
⁸ Department of Hepatology and Liver Transplantation, Royal Free Hospital NHS Foundation Trust, London, UK.
⁹ Institute of Liver Studies, King's College Hospital NHS Foundation Trust, Denmark Hill, London, UK.
¹⁰ Aneurin Bevan University Health Board, Newport, UK.
¹¹ Department of Hepatology, Forth Valley Royal Hospital, Larbert, UK.
¹² Department of Hepatology, Regional Liver Unit, Belfast Health and Social Care Trust, Belfast, UK.
¹³ Department of Hepatology, Glasgow Royal Infirmary, Glasgow, UK.
¹⁴ PBC Foundation, Edinburgh, UK.
¹⁵ Global Liver Institute, UK.
¹⁶ Liver Unit, Newcastle Upon Tyne Hospitals NHS Foundation Trust, Newcastle, UK.
¹⁷ NIHR Newcastle Biomedical Research Centre, Newcastle University, Newcastle, UK.
¹⁸ Institute of Applied Health Research, University of Birmingham, Birmingham, UK.
¹⁹ Department of Gastroenterology, St Mark's Hospital and Academic Institute, London, UK.
²⁰ Swansea University Medical School, Swansea, UK.

Abstract

Background & aims: Guidelines for the management of primary biliary cholangitis (PBC) were published by the British Society of Gastroenterology in 2018. In this study, we assessed adherence to these guidelines in the UK National Health Service (NHS).

Methods: All NHS acute trusts were invited to contribute data between 1 January 2021 and 31 March 2022, assessing clinical care delivered to patients with PBC in the UK.

Results: We obtained data for 8,968 patients with PBC and identified substantial gaps in care across all guideline domains. Ursodeoxycholic acid (UDCA) was used as first-line treatment in 88% of patients (n = 7,864) but was under-dosed in one-third (n = 1,964). Twenty percent of patients who were UDCA-untreated (202/998) and 50% of patients with inadequate UDCA response (1,074/2,102) received second-line treatment. More than one-third of patients were not assessed for fatigue (43%; n = 3,885) or pruritus (38%; n = 3,415) in the previous 2 years. Fifty percent of all patients with evidence of hepatic decompensation were discussed with a liver transplant centre (222/443). Appropriate use of second-line treatment and referral for liver transplantation was significantly better in specialist PBC treatment centres compared with non-specialist centres (p <0.001).

Conclusions: Poor adherence to guidelines exists across all domains of PBC care in the NHS. Although specialist PBC treatment centres had greater adherence to guidelines, no single centre met all quality standards. Nationwide improvement in the delivery of PBC-related healthcare is required.

Impact and implications: This population-based evaluation of primary biliary cholangitis, spanning four nations of the UK, highlights critical shortfalls in care delivery when measured across all guideline domains. These include the use of liver biopsy in diagnosis; referral practice for second-line treatment and/or liver transplant assessment; and the evaluation of symptoms, extrahepatic manifestations, and complications of cirrhosis. The authors therefore propose implementation of a dedicated primary biliary cholangitis care bundle that aims to minimise heterogeneity in clinical practice and maximise adherence to key guideline standards.

Keywords: Adherence; Autoimmune liver disease; Bezafibrate; Fenofibrate; Guideline; Liver transplantation; Obeticholic acid; Second-line therapy; Service evaluation; Surveillance.