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Current concepts: host-pathogen interactions in cystic fibrosis airways disease

Anthony C Tang1, Stuart E Turvey1, Marco P Alves2

  • 1Dept of Pediatrics, British Columbia Children's Hospital, Vancouver, British Columbia, Canada. Child and Family Research Institute, University of British Columbia, Vancouver, British Columbia, Canada. Division of Pediatric Respiratory Medicine, University Hospital, Bern, Switzerland. Clinical Research Group, Clinic for Pediatric Pneumology, Allergology and Neonatology, Hanover Medical School, Hanover, Germany. Cystic Fibrosis Research Group, Dept of Pediatrics I, University of Tübingen, Tübingen, Germany. Both authors contributed equally. Dept of Pediatrics, British Columbia Children's Hospital, Vancouver, British Columbia, Canada. Child and Family Research Institute, University of British Columbia, Vancouver, British Columbia, Canada. Division of Pediatric Respiratory Medicine, University Hospital, Bern, Switzerland. Clinical Research Group, Clinic for Pediatric Pneumology, Allergology and Neonatology, Hanover Medical School, Hanover, Germany. Cystic Fibrosis Research Group, Dept of Pediatrics I, University of Tübingen, Tübingen, Germany. Both authors contributed equally. Dept of Pediatrics, British Columbia Children's Hospital, Vancouver, British Columbia, Canada. Child and Family Research Institute, University of British Columbia, Vancouver, British Columbia, Canada. Division of Pediatric Respiratory Medicine, University Hospital, Bern, Switzerland. Clinical Research Group, Clinic for Pediatric Pneumology, Allergology and Neonatology, Hanover Medical School, Hanover, Germany. Cystic Fibrosis Research Group, Dept of Pediatrics I, University of Tübingen, Tübingen, Germany. Both authors contributed equally.

European Respiratory Review : an Official Journal of the European Respiratory Society
|September 2, 2014
PubMed

Insights

Cystic fibrosis (CF) airway disease involves chronic infections by multiple pathogens. This review explores how these CF pathogens infect, interact with, and evade the host immune system.

Area of Science:

  • Pulmonary Medicine
  • Microbiology
  • Immunology

Background:

  • Chronic infection and inflammation characterize cystic fibrosis (CF) airway disease.
  • The CF airway microenvironment supports colonization by diverse opportunistic pathogens.
  • Loss of cystic fibrosis transmembrane conductance regulator (CFTR) function leads to immune dysfunction, promoting pathogen persistence.

Purpose of the Study:

  • To review the current understanding of pathogen interactions in the CF airway.
  • To summarize how CF pathogens infect, interact with, and evade the CF host.

Main Methods:

  • Literature review of current research on CF pathogens.
  • Analysis of molecular identification techniques for microorganisms.
  • Examination of CF airway physiology and immune responses.

Main Results:

  • CF airway infections are polymicrobial, involving bacteria, viruses, and fungi.
  • CFTR dysfunction results in immune dysregulation, facilitating pathogen colonization.
  • Pathogens have evolved mechanisms to infect, persist, and evade host defenses in CF.

Conclusions:

  • Understanding pathogen-host interactions is crucial for managing CF lung disease.
  • Further research into microbial communities and host responses can inform therapeutic strategies.
  • The polymicrobial nature of CF infections requires a comprehensive approach to treatment.

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