The Role Of Fungi In Cystic Fibrosis: Actors Or Bystanders? - Two Cases Report

Authors

  • Isabel Morais Ribeiro Department of Pediatrics, Centro Materno-Infantil do Norte, Unidade Local de Saúde de Santo António https://orcid.org/0009-0006-4537-8332
  • Dora Fonseca Sousa Department of Pediatrics, Centro Materno-Infantil do Norte, Unidade Local de Saúde de Santo António
  • Ana Ascensão Matias Department of Pediatrics, Centro Materno-Infantil do Norte, Unidade Local de Saúde de Santo António https://orcid.org/0000-0002-4851-0486
  • Ana Lúcia Cardoso Cystic Fibrosis Reference Center, Centro Materno-Infantil do Norte, Unidade Local de Saúde de Santo António https://orcid.org/0000-0002-1575-5006
  • Telma Barbosa Pediatric Pulmonology Unit, Department of Pediatrics, Centro Materno-Infantil do Norte, Unidade Local de Saúde de Santo António

DOI:

https://doi.org/10.25753/BirthGrowthMJ.v35.i1.39803

Keywords:

antifungal agents, cystic fibrosis, fungi, hypersensitivity

Abstract

Introduction: Fungal infections increasingly contribute to morbidity in patients with cystic fibrosis (CF). Despite their rising incidence, there is considerable controversy surrounding the treatment and eradication of these infections in CF.
Case presentation: We present two CF patients: the first patient was largely asymptomatic despite intermittent detection of Scedosporium spp, while the second case experienced frequent pulmonary exacerbations (PEx) with persistent detection of both Aspergillus fumigatus (A. fumigatus) and Scedosporium spp. Both patients showed improvement after initiating the modulator therapy. Case 1 achieved complete eradication of fungal colonization, while case 2 remained sensitized to A. fumigatus but experienced fewer fungal exacerbations.
Discussion: These case reports underscore the variable clinical significance of fungal infections in CF and highlight the ongoing uncertainty regarding their role in disease progression. Further research is essential to elucidate their impact on CF and develop more tailored treatment strategies.

Downloads

Download data is not yet available.

References

King PT. The pathophysiology of bronchiectasis. Int J Chron Obstruct Pulmon Dis. 2009;4:411–419. doi: https://doi.org/10.2147/COPD.S6133.

Guo J, Garratt A, Hill A. Worldwide rates of diagnosis and effective treatment for cystic fibrosis. J Cyst Fibros. 2022;21(3):456–462. doi: https://doi.org/10.1016/j.jcf.2022.01.009.

Liu JC, Modha DE, Gaillard EA. What is the clinical significance of filamentous fungi positive sputum cultures in patients with cystic fibrosis? J Cyst Fibros. 2013;12(3):187–193. doi: https://doi.org/10.1016/j.jcf.2013.02.003.

Nguyen LD, Viscogliosi E, Delhaes L. The lung mycobiome: an emerging field of the human respiratory microbiome. Front Microbiol. 2015;6:89. doi: https://doi.org/10.3389/fmicb.2015.00089.

Singh A, Ralhan A, Schwarz C, et al. Fungal pathogens in CF airways: leave or treat? Mycopathologia. 2017;183(1):119–137. doi: https://doi.org/10.1007/s11046-017-0184-y.

Engel TG, Slabbers L, de Jong C, et al. Prevalence and diversity of filamentous fungi in the airways of cystic fibrosis patients: a Dutch multicentre study. J Cyst Fibros. 2019;18(2):221–226. doi: https://doi.org/10.1016/j.jcf.2018.11.012.

Bouchara JP, Le Govic Y, Kabbara S, et al. Advances in understanding and managing Scedosporium respiratory infections in patients with cystic fibrosis. Expert Rev Respir Med. 2020;14(3):259–273. doi: https://doi.org/10.1080/17476348.2020.1705787.

Breuer O, Schultz A, Garratt LW, et al. Aspergillus infections and progression of structural lung disease in children with cystic fibrosis. Am J Respir Crit Care Med. 2020;201(6):688–696. doi: https://doi.org/10.1164/rccm.201908-1585OC.

Balfour-Lynn IM, editor. Clinical guidelines: care of children with cystic fibrosis. 9th ed. London: Royal Brompton Hospital; 2023. Available from: https://www.rbht.nhs.uk/childrencf.

Francis NZ, Southern KW. Antifungal therapies for allergic bronchopulmonary aspergillosis in people with cystic fibrosis. Cochrane Database Syst Rev. 2022;9:CD002204. doi: https://doi.org/10.1002/14651858.CD002204.pub5.

Currie AJ, Main ET, Wilson HM, et al. CFTR modulators dampen Aspergillus-induced reactive oxygen species production by cystic fibrosis phagocytes. Front Cell Infect Microbiol. 2020;10:372. doi: https://doi.org/10.3389/fcimb.2020.00372.

Bessonova L, et al. Data from the US and UK cystic fibrosis registries support disease modification by CFTR modulation with ivacaftor. Thorax. 2018;73(8):731–740. doi: https://doi.org/10.1136/thoraxjnl-2017-210394.

Mehta AM, Lee I, Li G, et al. The impact of CFTR modulator triple therapy on type 2 inflammatory response in patients with cystic fibrosis. Allergy Asthma Clin Immunol. 2023;19:66. doi: https://doi.org/10.1186/s13223-023-00822-2.

Downloads

Published

2026-06-05

How to Cite

1.
Morais Ribeiro I, Fonseca Sousa D, Ascensão Matias A, Lúcia Cardoso A, Barbosa T. The Role Of Fungi In Cystic Fibrosis: Actors Or Bystanders? - Two Cases Report . BGMJ [Internet]. 2026 Jun. 5 [cited 2026 Jun. 6];35(1):41-6. Available from: https://revistas.rcaap.pt/bgmj/article/view/39803

Issue

Section

Clinical Case Reports

Most read articles by the same author(s)