ArticleERJ open research2025
Immunoglobulin A mucosal immunity in people with cystic fibrosis shortly after initiation of highly effective modulator therapy.
Article in ERJ open research, 2025. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 1 paper.
What it found
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The abstract states no effect estimate the extractor could read, or names no intervention and outcome on the map, so this paper lights no cell and moves no belief. It is still indexed, cited and linked below.
The trial behind it
Trials whose registry record cites this paper, or whose number appears in the abstract. A trial that started after this paper was published is citing it as background, not reporting it.
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Who cites it
1 citing paper in PubMed.
- Crosstalk between the microbiome and the mucosal immunoglobulin A system in the lung, in health and disease.Frontiers in cellular and infection microbiology · 2026Review
Corrections and comments
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Authors and funding
10 authors.
Funding
No grant is acknowledged in the PubMed record.
Abstract
Background: In the era of highly effective modulator therapies, pulmonary function, the number and severity of exacerbations and the quality of life of people with cystic fibrosis (CF) are improving significantly. The polymeric immunoglobulin receptor (pIgR)/IgA system, which is impaired in CF, protects the respiratory epithelium against harmful inhaled pathogens. The aims of this interventional prospective study were 1) to assess IgA mucosal immunity in bodily fluids from people with CF before and after elexacaftor/tezacaftor/ivacaftor (ETI) initiation, and 2) to investigate the mucosal IgA response in urine from people with CF compared with healthy controls. Methods: We quantified by ELISA the different agents comprising the pIgR/IgA system (total IgA, IgA1, IgA2, secretory IgA (S-IgA), secretory component of pIgR, and specific IgA against Results: Hyperactivation of the pIgR/IgA system was maintained under ETI, with no significant differences between total IgA, IgA1, IgA2, S-IgA nor secretory component levels in sputum, serum and urine from people with CF before and after ETI initiation, except for an increase in urine S-IgA after treatment. We demonstrate an activation of the mucosal immune system in the urinary tract of people with CF, with a higher level of total IgA (p=0.0141) compared with healthy controls. Conclusion: Even with highly effective modulator therapy, the pIgR/IgA system remains hyperactivated after ≥3 months of treatment, suggesting that at this time the local IgA immunity is always impaired.
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Registered trials
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