Current Concepts and Controversies in Innate Immunity of Cystic Fibrosis Lung Disease

Overview

Journal J Innate Immun

Publisher Karger

Specialty Allergy & Immunology

Date 2016 Jul 1

PMID 27362371

Citations 20

Authors

Anjali Ralhan

Julie Laval

Felipe Lelis

Marlene Ballbach

Charlotte Grund

Andreas Hector

Dominik Hartl

Affiliations

Soon will be listed here.

Abstract

Cystic fibrosis (CF) lung disease is characterized by chronic infection and inflammation. The inflammatory response in CF is dominated by the activation of the innate immune system. Bacteria and fungi represent the key pathogens chronically colonizing the CF airways. In response, innate immune pattern recognition receptors, expressed by airway epithelial and myeloid cells, sense the microbial threat and release chemoattractants to recruit large numbers of neutrophils into CF airways. However, neutrophils fail to efficiently clear the invading pathogens, but instead release harmful proteases and oxidants and finally cause tissue injury. Here, we summarize and discuss current concepts and controversies in the field of innate immunity in CF lung disease, facing the ongoing questions of whether inflammation is good or bad in CF and how innate immune mechanisms could be harnessed therapeutically.

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