Intestinal epithelium impairment in cystic fibrosis: What consequences in clinical practice?

doi:10.3748/wjg.121071

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World Journal of Gastroenterology

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1007-9327

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Baishideng Publishing Group Inc, 7041 Koll Center Parkway, Suite 160, Pleasanton, CA 94566, USA

Minireviews

Copyright: ©Author(s) 2026. This article is an open access article distributed under the terms and conditions of the Creative Commons Attribution-NonCommercial (CC BY-NC 4.0) license. No commercial re-use. See permissions. Published by Baishideng Publishing Group Inc.

World J Gastroenterol. Nov 14, 2026; 32(42): 121071
Published online Nov 14, 2026. doi: 10.3748/wjg.121071

Intestinal epithelium impairment in cystic fibrosis: What consequences in clinical practice?

Caroline Carlé, Marie Mittaine, Léa Roditis, Nolwenn Laborde, Frederick Barreau, Emmanuel Mas

Caroline Carlé, University of Toulouse, Department of Immunology Laboratory, Toulouse University Hospital Center, Toulouse 31000, Occitanie, France

Marie Mittaine, Léa Roditis, Nolwenn Laborde, Emmanuel Mas, University of Toulouse, Cystic Fibrosis Reference Center, Toulouse University Hospital, Toulouse 31000, Occitanie, France

Marie Mittaine, Léa Roditis, University of Toulouse, Department of Pediatric Pulmonology and Allergology, Toulouse University Hospital, Toulouse 31000, Occitanie, France

Nolwenn Laborde, Emmanuel Mas, University of Toulouse, Department of Gastroenterology, Hepatology, Nutrition and Inborn Errors of Metabolism, Toulouse University Hospital, Toulouse 31000, Occitanie, France

Frederick Barreau, Emmanuel Mas, University of Toulouse, INSERM UMR1220, INRAE, ENVT, Toulouse 31024, Occitanie, France

Author contributions: Carlé C, Barreau F and Mas E conceptualized the minireview; Carlé C wrote the paper and created the figures; Barreau F and Mas E provided the input in writing the paper; Mittaine M, Roditis L and Laborde N reviewed and edited the paper; Frederick Barreau and Emmanuel Mas made equal contributions.

AI contribution statement: AI tools were not used by the authors for this article.

Conflict-of-interest statement: There is no conflict of interest associated with any of the senior author or other coauthors contributed their efforts in this manuscript.

Corresponding author: Emmanuel Mas, Head, Professor, University of Toulouse, Department of Gastroenterology, Hepatology, Nutrition and Inborn Errors of Metabolism, Toulouse University Hospital, Toulouse 31000, Occitanie, France. mas.e@chu-toulouse.fr

Received: March 23, 2026
Revised: May 18, 2026
Accepted: August 10, 2026
Published online: November 14, 2026
Processing time: 188 Days and 9.8 Hours

Abstract

Cystic fibrosis (CF) results from the loss of function of the CF transmembrane conductance regulator protein, which is expressed at the apical membrane of various epithelia, including the digestive tract, and involved in chloride and bicarbonate transport. While modulators can improve pulmonary function, digestive symptoms remain a concern for patients with CF. Against this backdrop, it seems important to provide a current overview of research on intestinal inflammation in CF and a detailed analysis of the impact of this inflammation on each intestinal barrier compartment. It would also be useful to offer a description of the consequences of intestinal inflammation on associated complications, such as digestive cancers, and a comprehensive assessment of CF treatments and their impact on digestive manifestations. The different compartments of the intestinal barrier are impaired in CF (gut microbiota, mucus layer, intestinal epithelium, and immune cells), leading to chronic inflammation. This may explain the risk of colorectal cancer.

Keywords: Cystic fibrosis; Intestinal permeability; Microbiota; Mucus layer; Intestinal stem cells

Core Tip: Several reviews in the literature consider the role of and impact on the intestines in cystic fibrosis (CF) pathophysiology. The focus of such studies are, however, the gut-lung axis, the intestinal microbiota, the mucus layer, or electrolyte transport. This is the first review to take into account all aspects of the intestinal barrier (microbiota, mucus layer, intestinal epithelium, and immune system) that are altered in CF and may be involved in intestinal inflammation or colorectal cancer. The present review should therefore contribute to knowledge of intestinal pathophysiology in CF and inform the management of patients with gastrointestinal manifestations of CF.