Innate immunity in cystic fibrosis lung disease

被引：181

作者：

Hartl, D. ^{[1
]}

Gaggar, A. ^{[2
,3
]}

Bruscia, E. ^{[4
]}

Hector, A. ^{[1
]}

Marcos, V. ^{[5
]}

Jung, A. ^{[6
]}

Greene, C. ^{[7
]}

McElvaney, G. ^{[7
]}

Mall, M. ^{[8
,9
]}

Doering, G.

机构：

[1] Univ Tubingen, Dept Pediat 1, Tubingen, Germany

[2] Univ Alabama Birmingham, Dept Med, Birmingham, AL 35294 USA

[3] Univ Alabama Birmingham, Dept Physiol Biophys, Birmingham, AL USA

[4] Yale Univ, Sch Med, Dept Pediat, New Haven, CT 06510 USA

[5] Univ Munich, Dept Pediat, Munich, Germany

[6] Univ Childrens Hosp, Dept Resp Med, Zurich, Switzerland

[7] Beaumont Hosp, Royal Coll Surg Ireland, Educ & Res Ctr, Resp Res Div, Dublin 9, Ireland

[8] Heidelberg Univ, Div Pediat Pulmonol & Allergy, Heidelberg, Germany

[9] Heidelberg Univ, Cyst Fibrosis Ctr, Dept Pediat 3, Heidelberg, Germany

来源：

JOURNAL OF CYSTIC FIBROSIS | 2012年 / 11卷 / 05期

关键词：

Cystic fibrosis; Lung disease; Bacteria; Immunity; Innate immunity; Pattern recognition; Host defense; Chemokines; Toll-like receptors; Neutrophils; Macrophages; Phagocytosis; Migration; IL-17; PGP; Pseudomonas; TRANSMEMBRANE CONDUCTANCE REGULATOR; AIRWAY EPITHELIAL-CELLS; ALLERGIC BRONCHOPULMONARY ASPERGILLOSIS; TUMOR-NECROSIS-FACTOR; TOLL-LIKE RECEPTOR-2; PSEUDOMONAS-AERUGINOSA INFECTION; PATTERN-RECOGNITION RECEPTORS; HUMAN MONOCYTES DEMONSTRATION; POTENT PHAGOCYTIC-ACTIVITY; BINDING LECTIN MBL;

D O I：

10.1016/j.jcf.2012.07.003

中图分类号：

R56 [呼吸系及胸部疾病];

学科分类号：

摘要：

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. (C) 2012 European Cystic Fibrosis Society. Published by Elsevier B.V. All rights reserved.

引用

页码：363 / 382

页数：20

共 285 条

[1] Autophagy stimulation by rapamycin suppresses lung inflammation and infection by Burkholderia cenocepacia in a model of cystic fibrosis [J].