Elsevier

Journal of Cystic Fibrosis

Volume 11, Issue 5, September 2012, Pages 363-382
Journal of Cystic Fibrosis

Review
Innate immunity in cystic fibrosis lung disease

https://doi.org/10.1016/j.jcf.2012.07.003Get rights and content
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Abstract

Chronic lung disease determines the morbidity and mortality of cystic fibrosis (CF) patients. The pulmonary immune response in CF is characterized by an early and non-resolving activation of the innate immune system, which is dysregulated at several levels. Here we provide a comprehensive overview of innate immunity in CF lung disease, involving (i) epithelial dysfunction, (ii) pathogen sensing, (iii) leukocyte recruitment, (iv) phagocyte impairment, (v) mechanisms linking innate and adaptive immunity and (iv) the potential clinical relevance. Dissecting the complex network of innate immune regulation and associated pro-inflammatory cascades in CF lung disease may pave the way for novel immune-targeted therapies in CF and other chronic infective lung diseases.

Keywords

Cystic fibrosis
Lung disease
Bacteria
Immunity
Innate immunity
Pattern recognition
Host defense
Chemokines
Toll-like receptors
Neutrophils
Macrophages
Phagocytosis
Migration
IL-17
PGP
Chemokines
Pseudomonas

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