Inflammation in preschool cystic fibrosis is of mixed phenotype, extends beyond the lung and is differentially modified by CFTR modulators.

ABSTRACT:

Background

Early-life inflammation has long been recognised as a key pathophysiological process in the evolution of cystic fibrosis (CF) lung disease. Despite this, no CF-specific anti-inflammatory treatments have been developed. This is crucial even in the era of highly effective modulator therapy as recent evidence suggests that modulators alter, but may not fully resolve, pulmonary inflammation.

Methods

In this study, we used clinical microbiology data, high-dimensional flow cytometry and multiplex immunoassays to compare pulmonary (bronchoalveolar lavage (BAL)) and systemic immunity in 70 preschool children with CF and a total of 32 age-matched preschool controls.

Results

We show that inflammation in the early-life CF lung is characterised by innate cell infiltrat

SUBMITTER: Shanthikumar S 

PROVIDER: S-EPMC12322466 | biostudies-literature | 2025 Jun

REPOSITORIES: biostudies-literature