Evidence map›Paper›PMID 39293854›Full record

ReviewEuropean respiratory review : an official journal of the European Respiratory Society2024

Neutrophil serine proteases in cystic fibrosis: role in disease pathogenesis and rationale as a therapeutic target.

Marcus A Mall, Jane C Davies, Scott H Donaldson, Raksha Jain, James D Chalmers, Michal Shteinberg

Abstract readReview
In one paragraph

Review in European respiratory review : an official journal of the European Respiratory Society, 2024. The graph could read no effect estimate from its abstract, so it casts no vote on the map. Cited by 20 papers.

0numbers the graph read from it
0cells of the map it votes in
20citing papers in PubMed
–field-weighted citation impact
1 · What the graph read from it

What it found

Each row is one number read from the abstract, on the scale the paper reported it, with its interval. Left of the dashed line favours the treatment, right favours the comparator. Under each row is the sentence it came from. New to these charts? A ten-minute tutorial.

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.

2 · The registry

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.

Neither the registry nor the abstract names a trial number. If this is a trial report, that itself is worth knowing.

3 · Its place in the literature

Who cites it

20 citing papers in PubMed.

  1. The dipeptidyl peptidase 1 inhibition landscape in bronchiectasis and its future direction.European respiratory review : an official journal of the European Respiratory Society · 2026
    Review
  2. Review
  3. Article
  4. Article
  5. Article
  6. Evaluation of ATP12A and NFKBIZ as potential markers of inflammatory status in cystic fibrosis airway epithelial cells.Inflammation research : official journal of the European Histamine Research Society ... [et al.] · 2026
    Article
  7. Article
  8. Review
  9. Microbial and host innate immune factors affecting the persistence ofFrontiers in cellular and infection microbiology · 2026
    Review
  10. Article
  11. Article
  12. Article
  13. Neutrophil store-operated CaJournal of cystic fibrosis : official journal of the European Cystic Fibrosis Society · 2025
    Article
  14. Article
  15. Review
  16. Review
  17. Article
  18. Review
  19. Review
  20. Article
4 · The record

Corrections and comments

PubMed lists nothing against this paper. Absence here is not a guarantee, only a check that was made.

5 · Who and what money

Authors and funding

6 authors.

Marcus A MallDepartment of Pediatric Respiratory Medicine, Immunology and Critical Care Medicine and Cystic Fibrosis Center, Charité - Universitätsmedizin Berlin, corporate member of Freie Universität Berlin and Humboldt-Universität zu Berlin, Berlin, Germany marcus.mall@charite.de.ORCID https://orcid.org/0000-0002-4057-2199
Jane C DaviesNational Heart and Lung Institute, Imperial College London, London, UK.
Scott H DonaldsonDepartment of Medicine, Division of Pulmonary Diseases and Critical Care Medicine, The University of North Carolina at Chapel Hill, Chapel Hill, NC, USA.ORCID https://orcid.org/0000-0003-4802-0388
Raksha JainDepartment of Internal Medicine, University of Texas Southwestern Medical Center, Dallas, TX, USA.
James D ChalmersUniversity of Dundee, Dundee, UK.
Michal ShteinbergLady Davis Carmel Medical Center, Haifa, Israel.ORCID https://orcid.org/0000-0002-0432-3398

Funding

No grant is acknowledged in the PubMed record.

6 · The paper itself

Abstract

Chronic airway inflammation is a central feature in the pathogenesis of bronchiectasis (BE), which can be caused by cystic fibrosis (CFBE; hereafter referred to as CF lung disease) and non-CF-related conditions (NCFBE). Inflammation in both CF lung disease and NCFBE is predominantly driven by neutrophils, which release proinflammatory cytokines and granule proteins, including neutrophil serine proteases (NSPs). NSPs include neutrophil elastase, proteinase 3 and cathepsin G. An imbalance between NSPs and their antiproteases has been observed in people with CF lung disease and people with NCFBE. While the role of the protease/antiprotease imbalance is well established in both CF lung disease and NCFBE, effective therapies targeting NSPs are lacking. In recent years, the introduction of CF transmembrane conductance regulator (CFTR) modulator therapy has immensely improved outcomes in many people with CF (pwCF). Despite this, evidence suggests that airway inflammation persists, even in pwCF treated with CFTR modulator therapy. In this review, we summarise current data on neutrophilic inflammation in CF lung disease to assess whether neutrophilic inflammation and high, uncontrolled NSP levels play similar roles in CF lung disease and in NCFBE. We discuss similarities between the neutrophilic inflammatory profiles of people with CF lung disease and NCFBE, potentially supporting a similar therapeutic approach. Additionally, we present evidence suggesting that neutrophilic inflammation persists in pwCF treated with CFTR modulator therapy, at levels similar to those in people with NCFBE. Collectively, these findings highlight the ongoing need for new treatment strategies targeting neutrophilic inflammation in CF lung disease.

Indexed as

Cystic FibrosisCystic Fibrosis Transmembrane Conductance RegulatorInflammation MediatorsLungMolecular Targeted TherapyNeutrophilsAnimalsAnti-Inflammatory AgentsBronchiectasisHumansSerine ProteasesSerine Proteinase InhibitorsSignal TransductionAnti-Inflammatory AgentsCFTR protein, humanCystic Fibrosis Transmembrane Conductance RegulatorInflammation MediatorsSerine ProteasesSerine Proteinase Inhibitors

Identifiers

PMID39293854
PMCPMC11409056

What OpenQuestion holds

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Registered trials

None linked

Read under generation 80e0d062 · epoch 390. Bibliography from PubMed, PubMed Central and OpenAlex; grants from NIH RePORTER; trial links from ClinicalTrials.gov; estimates, votes and beliefs from the OpenQuestion graph.