What is the importance of classifying<i>Aspergillus</i>disease in cystic fibrosis patients?

Jones, Andrew M.; Horsley, Alex; Denning, David W.

doi:10.1586/17476348.2014.915751

Cited by 23 publications

(24 citation statements)

References 23 publications

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“…The classification of Aspergillus-related pulmonary disorders is constantly changing [70]. Inhalation of A. fumigatus spores leads to a spectrum of pulmonary manifestations in patients with CF.…”

Section: Discussionmentioning

confidence: 99%

Prevalence of Aspergillus sensitization and allergic bronchopulmonary aspergillosis in cystic fibrosis: systematic review and meta‐analysis

Maturu

Agarwal

2015

Clin Experimental Allergy

141

110

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Section: Discussionmentioning

confidence: 99%

Prevalence of Aspergillus sensitization and allergic bronchopulmonary aspergillosis in cystic fibrosis: systematic review and meta‐analysis

Maturu

Agarwal

2015

Clin Experimental Allergy

141

110

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“…A. fumigatus is a saprophytic spore-forming mould found widely in the environment [32]. The spores are 2–4 mm in diameter, and thousands of these spores are inhaled daily [38].…”

Section: Introductionmentioning

confidence: 99%

Pathogenesis of Fungal Infections in Cystic Fibrosis

Williams

Ranjendran

Ramage

2016

Curr Fungal Infect Rep

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For a long time, the microbiology of cystic fibrosis has been focussed on Pseudomonas aeruginosa and associated Gram-negative pathogens. An increasing body of evidence has been compiled demonstrating an important role for moulds and yeasts within this complex patient group. Whether or not fungi are active participants, spectators or transient passersby remain to be elucidated. However, functionally, they do appear to play a contributory role in pathogenesis, albeit we do not know if this is a direct or indirect effect. The following review examines some of the key evidence for the role of fungi in CF pathogenesis.

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“…18), where the colonization by the fungus is common and may lead to fungal sensitization, bronchitis and allergic broncho-pulmonary aspergillosis (ABPA)19 as well as worse forced expiratory volume in the first second (FEV1) (ref. 20). In CF patients, the expression of IL-9 and IL-9R is increased and is associated with mucus overproduction, but whether and how IL-9 contributes to immunity and pathology in response to the fungal infection in CF is not known.…”

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confidence: 99%

A mast cell-ILC2-Th9 pathway promotes lung inflammation in cystic fibrosis

et al. 2017

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T helper 9 (Th9) cells contribute to lung inflammation and allergy as sources of interleukin-9 (IL-9). However, the mechanisms by which IL-9/Th9 mediate immunopathology in the lung are unknown. Here we report an IL-9-driven positive feedback loop that reinforces allergic inflammation. We show that IL-9 increases IL-2 production by mast cells, which leads to expansion of CD25+ type 2 innate lymphoid cells (ILC2) and subsequent activation of Th9 cells. Blocking IL-9 or inhibiting CD117 (c-Kit) signalling counteracts the pathogenic effect of the described IL-9-mast cell-IL-2 signalling axis. Overproduction of IL-9 is observed in expectorates from cystic fibrosis (CF) patients, and a sex-specific variant of IL-9 is predictive of allergic reactions in female patients. Our results suggest that blocking IL-9 may be a therapeutic strategy to ameliorate inflammation associated with microbial colonization in the lung, and offers a plausible explanation for gender differences in clinical outcomes of patients with CF.

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What is the importance of classifyingAspergillusdisease in cystic fibrosis patients?

Cited by 23 publications

References 23 publications

Prevalence of Aspergillus sensitization and allergic bronchopulmonary aspergillosis in cystic fibrosis: systematic review and meta‐analysis

Prevalence of Aspergillus sensitization and allergic bronchopulmonary aspergillosis in cystic fibrosis: systematic review and meta‐analysis

Pathogenesis of Fungal Infections in Cystic Fibrosis

A mast cell-ILC2-Th9 pathway promotes lung inflammation in cystic fibrosis

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