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Innate immunity in cystic fibrosis lung disease

Year: 2012
Type: review
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.
DOI: 10.1016/j.jcf.2012.07.003
Authors: D. Hartl, A. Gaggar, E. Bruscia, A. Hector, V. Marcos, A. Jung, C. Greene, G. McElvaney, M. Mall, G. Döring
Institutions: University of Tübingen, University of Alabama at Birmingham, Yale University, Ludwig-Maximilians-Universität München, University Children's Hospital Zurich, Royal College of Surgeons in Ireland, Beaumont Hospital, Heidelberg University
Language: English
Field: Medicine
Open Access status: bronze
Awards: G1717694172, G4114538474, G448742297, G4559714697, A Novel Proteolytic System of Pulmonary Inflammation, G7993178521
OpenAlex ID: W2117017587
FWCI: 3.312
Cites: 284
Cited by: 242
Related to: 10