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Fungal Pathogens in CF Airways: Leave or Treat?

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Abstract

Chronic airway infection plays an essential role in the progress of cystic fibrosis (CF) lung disease. In the past decades, mainly bacterial pathogens, such as Pseudomonas aeruginosa, have been the focus of researchers and clinicians. However, fungi are frequently detected in CF airways and there is an increasing body of evidence that fungal pathogens might play a role in CF lung disease. Several studies have shown an association of fungi, particularly Aspergillus fumigatus and Candida albicans, with the course of lung disease in CF patients. Mechanistically, in vitro and in vivo studies suggest that an impaired immune response to fungal pathogens in CF airways renders them more susceptible to fungi. However, it remains elusive whether fungi are actively involved in CF lung disease pathologies or whether they rather reflect a dysregulated airway colonization and act as microbial bystanders. A key issue for dissecting the role of fungi in CF lung disease is the distinction of dynamic fungal–host interaction entities, namely colonization, sensitization or infection. This review summarizes key findings on pathophysiological mechanisms and the clinical impact of fungi in CF lung disease.

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Acknowledgements

This work was supported by the German Research Foundation (DFG to A.H.; DFG, SFB/CRC685 at Tübingen to D.H.), Christiane-Herzog-Stiftung (Christiane-Herzog-Award to A.H.) and funding of the University of Tübingen (Junior Research Group Program/IZKF to A.H.). We would like to thank Mr. Peter-Michael Weber for his excellent work on the illustration.

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  1. Department of Pediatrics, Pediatric Infectiology, Immunology and Cystic Fibrosis, Children’s Hospital, University of Tübingen, Hoppe-Seyler-Str. 1, 72076, Tübingen, Germany

    A. Singh, A. Ralhan, D. Hartl & A. Hector

  2. Department of Pediatric Pneumology and Immunology, Cystic Fibrosis Center Berlin/Charité, University of Berlin, Berlin, Germany

    C. Schwarz

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  1. A. Singh
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Singh, A., Ralhan, A., Schwarz, C. et al. Fungal Pathogens in CF Airways: Leave or Treat?. Mycopathologia 183, 119–137 (2018). https://doi.org/10.1007/s11046-017-0184-y

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