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Annotated abstract

The changing face of inflammation following CFTR modulation: identifying new phenotypes of innate immunity

thoraxjnl · 2025-08-15 · canonical JSON source

6 visible annotations · policy: published · automated confidence ≥ 75.00%

Document resource

Triple combination therapy for people with cystic fibrosis (pwCF) restores the activity of defective cystic fibrosis transmembrane conductance regulator (CFTR) proteins. For those fortunate to receive this therapy, it has fundamentally changed the nature of the disease.1 CFTR modulators markedly improve clinical outcomes and symptoms, reversing or alleviating pathologies such as mucous plugging and overly acidic small intestine pH. However, other manifestations of disease persist (established bronchiectasis, chronic airway infections with organisms such as Pseudomonas aeruginosa). Optimal management of pwCF depends on continued accumulation of knowledge regarding how CFTR therapy changes CF disease. 2