WS08.02 Change in hepatobiliary ultrasound measures, incl. shear wave elastography, after introduction of elexacaftor/tezacaftor/ivacaftor: results from 12-month follow-up in the Danish cystic fibrosis cohort

Objectives: A new era in cystic fibrosis (CF) since the introduction of CF transmembrane conductance regulator modulators with elexacaftor/tezacaftor/ivacaftor (ETI) is marked by significantly improved lung function and nutritional status. However, the impact of ETI on the liver remains poorly characterized, as advanced liver disease was an exclusion criterion in clinical trials. Clinical experience from the Danish CF cohort has shown ETI to be generally well-tolerated in term of liver biomarkers. The objective of this study is to assess hepatobiliary ultrasonographic changes before and twelve months after the initiation of ETI. Methods: All non-transplanted Danish CF patients aged ≥12 years with at least one F508del variant, started on ETI regardless of liver involvement, are included. Ultrasound was performed within three months before treatment initiation and at follow-up twelve months after. The liver size, parenchyma, and edge are described alongside the portal vein flow and pattern. The presence of focal changes, fibrosis, and/or ascites is recorded, and both the appearance of the biliary tract, gall bladder, and spleen is documented. Shear wave elastography is performed on a subgroup of fasting patients before and twelve months after treatment initiation. Preliminary results: 331 patients (48.6% female) were initiated on ETI. The median age was 27 years (range 12;70). Results of hepatobiliary changes on ultrasound, including a subgroup with shear wave elastography, will be available after data collection is completed in March 2023 before the ECFS conference. Conclusions: Given the success of ETI treatment in improving pulmonary and nutritional status, and anticipated effects on life expectancy, the future focus of care will include other causes of morbidity and mortality, such as liver disease. Findings of the present study will provide important knowledge on CF liver disease after twelve months of ETI treatment.