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Cystic Fibrosis-Related Gut Dysbiosis: A Systematic Review

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Abstract

Background and Aims

Cystic Fibrosis (CF) is associated with gut dysbiosis, local and systemic inflammation, and impaired immune function. Gut microbiota dysbiosis results from changes in the complex gut milieu in response to CF transmembrane conductance regulator (CFTR) dysfunction, pancreatic malabsorption, diet, medications, and environmental influences. In several diseases, alteration of the gut microbiota influences local and systemic inflammation and disease outcomes. We conducted a systematic review of the gut microbiota in CF and explored factors influencing dysbiosis.

Methods

An electronic search of three databases was conducted in January 2019, and re-run in June 2021. Human, animal, and in vitro studies were included. The primary outcome was differences in the gut microbiota between people with CF (pwCF) and healthy controls. Secondary outcomes included the relationship between the gut microbiota and other factors, including diet, medication, inflammation, and pulmonary function in pwCF.

Results

Thirty-eight studies were identified. The literature confirmed the presence of CF-related gut dysbiosis, characterized by reduced diversity and several taxonomic changes. There was a relative increase of bacteria associated with a pro-inflammatory response coupled with a reduction of those considered anti-inflammatory. However, studies linking gut dysbiosis to systemic and lung inflammation were limited. Causes of gut dysbiosis were multifactorial, and findings were variable. Data on the impact of CFTR modulators on the gut microbiota were limited.

Conclusions

CF-related gut dysbiosis is evident in pwCF. Whether this influences local and systemic disease and is amenable to interventions with diet and drugs, such as CFTR modulators, requires further investigation.

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Acknowledgments

Artwork by Laura & John Caley ©.

Funding

This work was supported by the Cystic Fibrosis Trust [SRC 012].

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Authors and Affiliations

  1. Leeds Institute of Medical Research, St James’s University Hospital, Clinical Sciences Building, Leeds, LS9 7TF, UK

    L. R. Caley & D. G. Peckham

  2. Nutrition, Health & Environment, Leeds Beckett University, Leeds, UK

    H. White

  3. Wellcome Sanger Institute, Cambridge, UK

    M. C. de Goffau

  4. Tytgat Institute for Liver and Intestinal Research, Amsterdam University Medical Centers, Amsterdam, The Netherlands

    M. C. de Goffau

  5. Molecular Immunity Unit, Department of Medicine, University of Cambridge, Cambridge, UK

    R. A. Floto

  6. Cambridge Centre for Lung Infection, Royal Papworth Hospital, Cambridge, UK

    R. A. Floto

  7. Department of Veterinary Medicine, University of Cambridge, Cambridge, UK

    J. Parkhill

  8. Department of Immunology and Pathology, Central Clinical School, Monash University, Melbourne, Australia

    B. Marsland

  9. Department of Respiratory Medicine, Leeds Teaching Hospitals NHS Trust, Leeds, UK

    D. G. Peckham

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LRC contributed toward conceptualization, data curation, formal analysis, methodology, project administration, writing—original draft and review & editing; MCG contributed toward formal analysis, methodology, validation, writing—review & editing. AF contributed toward writing—review & editing. JP contributed toward writing—review & editing. BM contributed toward writing—review & editing. HW contributed toward data curation, formal analysis, methodology, supervision, validation, writing—original draft and review & editing and supervision. DGP contributed toward conceptualization data curation, formal analysis, methodology, supervision, validation, and writing—original draft and review & editing.

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Caley, L.R., White, H., de Goffau, M.C. et al. Cystic Fibrosis-Related Gut Dysbiosis: A Systematic Review. Dig Dis Sci 68, 1797–1814 (2023). https://doi.org/10.1007/s10620-022-07812-1

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