Skip Navigation LinksHome > January 2014 - Volume 21 - Issue 1 > The role of neutrophils in cystic fibrosis
Current Opinion in Hematology:
doi: 10.1097/MOH.0000000000000009
MYELOID BIOLOGY: Edited by David C. Dale

The role of neutrophils in cystic fibrosis

Gifford, Alison M.a; Chalmers, James D.b

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Abstract

Purpose of review

Neutrophils are known to dominate the pulmonary inflammatory process observed in cystic fibrosis (CF). An enduring paradox is how these large numbers of neutrophils fail to eradicate colonizing bacteria. Major advances in our understanding of neutrophil dysfunction in CF and its effect on the innate immune system are leading to advances in our understanding of the pathophysiology and leading directly to new therapies.

Recent findings

New mechanisms of neutrophil dysfunction have been described in CF including disabled cystic fibrosis transmembrane conductance regulator recruitment to phagosomes and novel mechanisms of protease-induced neutrophil dysfunction. Neutrophil elastase has been shown to be present in the airway very early in life in CF patients, and appears a biomarker of disease progression, predicting lung function decline and bronchiectasis. Elastase has also been shown to induce a pro-inflammatory state of senescence in bronchial epithelial cells in vitro and potentially in vivo. Inhibitors of neutrophil elastase are now entering clinical trials with promising results. New avenues of CF therapeutics are being explored including novel macrolides, CXCR2 antagonists and exogenous opsonins.

Summary

This article reviews the past 12 months of research that contributes to our understanding of the role of neutrophils and immune dysfunction in CF.

© 2013 Wolters Kluwer Health | Lippincott Williams & Wilkins

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