Cystic fibrosis (CF) is a common autosomal recessive disease due to mutations of the gene encoding the cystic fibrosis transmembrane conductance regulator.The objective of this anal. was to investigate the clin. characteristics of T2 inflammation in CF, whether T2 inflammation in CF is associated with worse outcomes, and how CFTR modulator therapy influences T2 biomarker levels.T2 endotype exists within CF characterized by increased obstructive pulmonary disease, and distinct phenotypic signature of increased allergic disease, infections, and burden of CF complications.Most importantly, we also demonstrate for the first time an increased risk of death among CF subjects with T2 inflammatory signatures compared to CF subjects lacking significant T2 inflammation.Concordantly, we found that CFTR modulator therapy was associated with IgE reductions, suggesting a complex relationship between CFTR function and T2 inflammation.Understanding how common CF pathogens influence the T2 signature and whether T2 inflammation contributes to bacterial or fungal colonization may provide insights into disease pathogenesis.Although IgE and AEC are commonly performed as annual laboratory tests for CF patients, these tests may not completely reflect T2 inflammation in the lung.Further studies identifying modifiers of T2 inflammation in CF may have prognostic and therapeutic importance in this vulnerable population.
