P105 Addressing liver disease in cystic fibrosis: insights from a local audit

<h3></h3> Liver abnormalities are common in people with cystic fibrosis (pwCF), with approximately 40% displaying some form of liver dysfunction. Of these, 5–10% may experience clinically significant health impacts.¹ NICE guidelines recommend annual clinical assessments and liver function blood tests for pwCF. If abnormalities are detected, a liver ultrasound scan (USS) and consideration of ursodeoxycholic acid (UDCA) treatment are advised. ECFS CFLD guidelines suggest starting UDCA at 20 mg/kg,² but a range of 10–20 mg/kg has been reported to have no difference in outcome.³ All pwCF registered at an adult CF centre as of October 2023 were identified. A retrospective chart review of the electronic patient record system (EPR) was completed, and data collected on demographics, date of last liver USS, diagnosis of CF-related liver disease (CFLD) or other liver conditions and use of CFTR modulators. Management was compared to national and international guidelines. n=612 patients were identified. Those who had passed away were excluded, therefore n=609 were included in the audit: male n=312 (51.2%). n=470 (77.2%) had a liver USS result recorded on EPR. Additionally, n=264 (43.3%) had an elastography result. n=97 (15.9%) had a formal diagnosis of CFLD. UDCA was prescribed to n=100 (16.4%), of whom n=69, had a formal CFLD diagnosis, while n=31 were on UDCA and did not have a diagnosis of CFLD, n=3 (9.6%) had UCDA initiated secondary to gallstones and n=28 (90.4%) for other childhood liver enzyme abnormalities. Of the latter one patient had hepatitis B and n=12 had hepatic steatosis. Of the patients on UDCA, n=33 (33%) were receiving doses outside the recommendations with a range of 2.9 – 28.6 mg/kg/day. Of these, n=26 (78.8%) had remained on paediatric dosing and n=7 (21.2%) had recent Kaftrio associated weight gain. Importantly, n=42 with a diagnosis of CLFD were not being reviewed in our CF hepatology clinic. This audit highlights a high prevalence of liver abnormalities in pwCF and the challenges in early CFLD diagnosis. Given the lack of diagnostic non-invasive tests for CFLD, liver enzyme abnormalities and changes in echogenicity often prompt initiation of UDCA, however, other causes of liver disease should also be considered. Additionally, UCDA dosing should be reviewed regularly, and dose adjustments made for paediatric patients as they age and for those on Kaftrio. Future efforts should focus on aligning treatment practices with established guidelines and enhancing the monitoring process to prevent the progression of liver disease in this population. <h3>References</h3> Toledano MB, Mukherjee SK, Howell J, Westaby D, Khan SA, Bilton D, Simmonds NJ. The emerging burden of liver disease in cystic fibrosis patients: a UK nationwide study.<i> PLoS One</i>. 2019 Apr 4;<b>14</b>(4):e0212779. doi: 10.1371/journal.pone.0212779. PMID: 30947265; PMCID: PMC6448894. Debray D, Kelly D, Houwen R, Strandvik B, Colombo C. Best practice guidance for the diagnosis and management of cystic fibrosis-associated liver disease. <i>Journal of Cystic Fibrosis</i>. 2011;<b>10</b>(Suppl 2):S29-S36. doi:10.1016/j.jcf.2011.09.012. Cheng K, Ashby D, Smyth RL. Ursodeoxycholic acid for cystic fibrosis-related liver disease. <i>Cochrane Database Syst Rev</i>. 2017 Sep 11;<b>9</b>(9):CD000222. doi: 10.1002/14651858.CD000222.pub4. PMID: 28891588; PMCID: PMC6483662.