title: Host-pathogen interplay in the respiratory environment of cystic fibrosis.
abstract: Significant advances have been made in the understanding of disease progression in cystic fibrosis (CF), revealing a complex interplay between host and pathogenic organisms. The diverse CF microbiota within the airway activates an aberrant immune response that is ineffective in clearing infection. An appreciation of how the CF host immune system interacts with these organisms is crucial to understanding the pathogenesis of CF pulmonary disease. Here we discuss the microbial complexity present in the lungs of individuals with CF, review emerging concepts of innate and adaptive immune responses to pathogens that chronically inhabit the CF lung, and discuss therapies that target the aberrant inflammatory response that characterizes CF. A greater understanding of the underlying mechanisms will shed light on pathogenesis and guide more targeted therapies in the future that serve to reduce infection, minimize lung pathology, and improve the quality of life for patients with CF.
authors: Mucosal Immunology & Biology Research Center, Pediatrics, Harvard Medical School, Massachusetts General Hospital for Children , Charlestown, MA, U.S.A.
authors: Lael M
authors: LM
authors: Yonker
authors: Infections and Cystic Fibrosis Unit, Division of Immunology, Transplantation and Infectious Diseases, IRCCS San Raffaele Scientific Institute, Milano, Italy.
authors: Cristina
authors: C
authors: Cigana
authors: Mucosal Immunology & Biology Research Center, Pediatrics, Harvard Medical School, Massachusetts General Hospital for Children , Charlestown, MA, U.S.A.
authors: Bryan P
authors: BP
authors: Hurley
authors: Infections and Cystic Fibrosis Unit, Division of Immunology, Transplantation and Infectious Diseases, IRCCS San Raffaele Scientific Institute, Milano, Italy.
authors: Alessandra
authors: A
authors: Bragonzi
mesh_terms: D056704:Adaptive Immunity / Q000502:physiology*; D003550:Cystic Fibrosis / Q000276:immunology* / Q000382:microbiology* / Q000473:pathology; D006801:Humans; D012141:Respiratory Tract Infections / Q000209:etiology* / Q000473:pathology / Q000628:therapy
keywords: Cystic fibrosis; Host–pathogen interaction; Innate and adaptive immunity; Lung disease; Microbiota