Alterations in lipids after initiation of highly effective modulators in people with cystic fibrosis

Area of Science:

• Cardiology
• Pulmonology
• Pharmacology

Background:

• Cardiovascular disease (CVD) risk may be altered in people with cystic fibrosis (pwCF) due to highly effective modulator therapy (HEMT).
• Understanding the impact of HEMT on lipid profiles is crucial for managing CVD risk in pwCF.

Purpose of the Study:

• To investigate the effect of HEMT (ivacaftor or elexacaftor/tezacaftor/ivacaftor) on lipid profiles in pwCF.
• To determine if HEMT influences total cholesterol (TC), LDL, HDL, and TC/HDL ratio.

Main Methods:

• Retrospective analysis of lipid levels before and after HEMT initiation in 41 adult pwCF.
• Paired t-tests and linear mixed models were used to analyze changes in lipid parameters.

Main Results:

• Significant increases observed in TC, LDL, and TC/HDL ratio post-HEMT initiation.
• HDL levels remained unchanged.
• CF liver disease was associated with blunted lipid changes, while a family history of CVD risk factors correlated with accentuated lipid increases.

Conclusions:

• HEMT is associated with adverse lipid changes in pwCF.
• Consideration of lipid screening in pwCF on HEMT is warranted, particularly in those with specific risk factors like CF liver disease or family history of CVD.