title: Current Concepts and Controversies in Innate Immunity of Cystic Fibrosis Lung Disease.
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.
authors.affiliation: Department of Pediatrics I, University of Tübingen, Tübingen, Germany.
authors.forename: Anjali
authors.initials: A
authors.lastname: Ralhan
authors.forename: Julie
authors.initials: J
authors.lastname: Laval
authors.forename: Felipe
authors.initials: F
authors.lastname: Lelis
authors.forename: Marlene
authors.initials: M
authors.lastname: Ballbach
authors.forename: Charlotte
authors.initials: C
authors.lastname: Grund
authors.forename: Andreas
authors.initials: A
authors.lastname: Hector
authors.forename: Dominik
authors.initials: D
authors.lastname: Hartl
mesh_terms: D000818:Animals; D018925:Chemokines / Q000378:metabolism; D003550:Cystic Fibrosis / Q000276:immunology*; D006801:Humans; D007113:Immunity, Innate; D007239:Infections / Q000276:immunology*; D007249:Inflammation / Q000276:immunology*; D008168:Lung / Q000276:immunology* / Q000473:pathology; D055370:Lung Injury / Q000276:immunology*; D020556:Neutrophil Infiltration; D051192:Receptors, Pattern Recognition / Q000378:metabolism; D012008:Recurrence
chemical_list: D018925:Chemokines; D051192:Receptors, Pattern Recognition