Abstract
Cystic fibrosis (CF), caused by mutations in the cystic fibrosis
transmembrane conductance regulator (CFTR) gene, is the most common
inherited life-limiting disease in North European people affecting 90,000
people worldwide. Progressive lung damage caused by recurrent infection
and chronic airway inflammation is the major determinant of survival with
a median age at death of 29 years. Approximately 60% of CF patients are
infected with Aspergillus fumigatus, a ubiquitous environmental fungus,
and its presence has been associated with accelerated lung function
decline. Half of the patients infected with Aspergillus are <18 years of age.
Yet, time of acquisition of this fungus and determinants of CF-related
Aspergillus disease severity and progression are not known.
CFTR expression has been demonstrated in cells of the innate and adaptive
immune system and has shown to be critical for normal function. Research
delineating the role of CFTR-deficient phagocytes in Aspergillus persistence
and infection in the CF lung, has only recently received attention. In this
concise review we aim to present the current understanding with respect to
when people with CF acquire infection with A. fumigatus and antifungal
immune responses by CF immune cells.
Original language | English |
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Pages (from-to) | S118-S126 |
Number of pages | 9 |
Journal | Medical Mycology |
Volume | 57 |
Issue number | Suppl. 2 |
Early online date | 28 Feb 2019 |
DOIs | |
Publication status | Published - 1 Apr 2019 |
Bibliographical note
Invited Review.AB and AW are supported by the Wellcome Trust Strategic Award in Medical Mycology and Fungal Immunology (grant 097377). AW is supported by the MRC Centre for Medical Mycology (grant MR/N006364/1) at the University of Aberdeen.
Keywords
- aspergillosis
- aspergillus fumigatus
- cystic fibrosis
- colonisation
- innate immunity
- colonization
- Aspergillus fumigatus