Modulation of immune responses by elexacaftor/tezacaftor/ivacaftor therapy in cystic fibrosis: data from a compassionate use program.

ABSTRACT:

Background

Elexacaftor-tezacaftor-ivacaftor (ETI) improves clinical outcomes in people with Cystic Fibrosis (pwCF), with possible anti-inflammatory properties. However, the molecular mechanisms underlying these effects remain unclear. This study investigates ETI's anti-inflammatory and immunomodulatory activity, focusing on essential signaling pathways.

Methods

Forty-nine pwCF were followed for 24 months. PwCF underwent clinical and pulmonary function assessment along with sweat test chloride measurement. Blood samples were analyzed for red and white blood cell counts and C-reactive protein (CRP). Plasma cytokines were quantified and phospho-kinase arrays and western blotting were used to assess protein phosphorylation in peripheral blood mononuclear cells (PBMC) from pwCF a

SUBMITTER: Lucca F 

PROVIDER: S-EPMC12911138 | biostudies-literature | 2026 Jan

REPOSITORIES: biostudies-literature