Effects of elexacaftor/tezacaftor/ivacaftor on liver fibrosis markers in adults with cystic fibrosis

Daniel H Tewkesbury, Jennifer Scott, Peter J Barry, Rowland J Bright-Thomas, Karen Piper Hanley, Varinder Athwal, Andrew M Jones

Research output: Contribution to journalArticlepeer-review

Abstract

Background: There are limited studies to date on the effects of elexacaftor/tezacaftor/ivacaftor (E/T/I) on markers of liver fibrosis in adults with cystic fibrosis (CF). This study aims to analyse changes in makers of liver fibrosis before and after initiation of E/T/I in CF adults. Methods: Outcome measures of liver fibrosis, including liver stiffness measurement (LSM) using FibroScan, AST-to-platelet-ratio index (APRI) and gamma-GT-to-platelet-ratio (GPR) were available in 74 CF adults following initiation of E/T/I. This was compared to historical data collected in 2018 prior to UK availability of E/T/I. Results: The median duration of E/T/I therapy at the time liver fibrosis markers were repeated was 21 (IQR: 17–25) months. There was an increase in APRI from historical measurement to follow-up but no change in LSM or GPR. There were no differences in change in fibrosis markers according to CF liver disease (CFLD) status, although those with a raised LSM at baseline (>6.8 kPa) (n = 14) had a significant reduction in LSM from historical measurement to follow-up versus those with a normal historical value (-3.3 kPa vs 0.25 kPa, p < 0.01). Conclusions: Apart from APRI, we found no changes in liver fibrosis outcomes after initiation of E/T/I in adults with CF. Those with a historical diagnosis of CFLD had no significant worsening or improvement of liver fibrosis markers. We did observe a reduction in LSM in those with liver nodularity, with an initial highest result suggesting a potential positive treatment effect of E/T/I in this category of those with severe CFLD.

Original languageEnglish
JournalJournal of Cystic Fibrosis
Early online date19 Sept 2023
DOIs
Publication statusE-pub ahead of print - 19 Sept 2023

Keywords

  • CFTR modulator therapy
  • Cystic fibrosis liver disease
  • Liver fibrosis

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