Author information

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