Effect of elexacaftor/tezacaftor/ivacaftor on systemic inflammation in cystic fibrosis.

ABSTRACT:

Background

Despite significant clinical improvements, there is evidence of persisting airway inflammation in people with cystic fibrosis (CF) established on elexacaftor/tezacaftor/ivacaftor (ETI) therapy. As CF is a multi-system disease, systemic immune profiles can reflect local inflammation within the lungs and other organs. Understanding systemic inflammation after ETI therapy may reveal important translational insights. This study aims to profile systemic inflammatory changes and relate these to the well-documented improvements observed with ETI therapy.

Methods

We conducted a single-centre longitudinal study with 57 CF subjects initiating ETI therapy. All participants were Phe508del homozygous or Phe508del/minimal function. Blood samples were collected pre-ETI and 3-12

SUBMITTER: Maher RE 

PROVIDER: S-EPMC12421123 | biostudies-literature | 2025 Aug

REPOSITORIES: biostudies-literature