Real world study on elexacaftor-tezacaftor-ivacaftor impact on cholesterol levels in adults with cystic fibrosis

Pharmacotherapy. 2024 Mar;44(3):231-240. doi: 10.1002/phar.2903. Epub 2024 Jan 12.

Abstract

Introduction: The introduction of the highly effective modulator therapy elexacaftor-tezacaftor-ivacaftor (ETI) has revolutionized the care of persons with cystic fibrosis (PwCF) with major improvements seen in lung function and body mass index. The effects of ETI therapy in real-world cohorts on other parameters such as cholesterol levels are largely unknown.

Methods: A single-center, retrospective chart review study was conducted to assess the change in lipid panels before and after ETI initiation. The study investigated total cholesterol (TC), low-density lipoprotein cholesterol (LDL-C), high-density lipoprotein cholesterol (HDL-C), and triglyceride levels using both a univariate and multivariate mixed-effects model to evaluate the change after initiation of ETI in a cohort of PwCF.

Results: There were 128 adult PwCF included in the analysis. Statistically significant changes were seen in both univariate and multivariate analyses for TC, LDL-C, and HDL-C. On multivariate analysis, TC increased by an average of 15.0 mg/dL after ETI initiation (p < 0.0001), LDL-C increased by an average of 9.3 mg/dL (p < 0.001), and HDL-C increased by an average of 3.8 mg/dL (p < 0.001) after ETI initiation.

Conclusion: In this real-world cohort of PwCF, cholesterol parameters increased after initiation with ETI therapy. Further consideration may need to be given for PwCF in regards to screening for cardiometabolic risk factors as PwCF age as well as the potential need for cholesterol-lowering therapies.

Keywords: cholesterol; cystic fibrosis; elexacaftor; ivacaftor; tezacaftor.

MeSH terms

  • Adult
  • Aminophenols*
  • Benzodioxoles*
  • Cholesterol, LDL
  • Cystic Fibrosis* / drug therapy
  • Humans
  • Indoles*
  • Mutation
  • Pyrazoles*
  • Pyridines*
  • Pyrrolidines*
  • Quinolones*
  • Retrospective Studies

Substances

  • elexacaftor
  • ivacaftor
  • tezacaftor
  • Cholesterol, LDL
  • Benzodioxoles
  • Indoles
  • Aminophenols
  • Pyrazoles
  • Pyridines
  • Pyrrolidines
  • Quinolones