Changing paradigms in the treatment of gastrointestinal complications of cystic fibrosis in the era of cystic fibrosis transmembrane conductance regulator modulators

Cystic fibrosis (CF) - although primarily a lung disease - also causes a variety of gastrointestinal manifestations which are important for diagnosis, prognosis and quality of life. All parts of the gastrointestinal tract can be affected by CF. Besides the well-known pancreatic insufficiency, gastroesophageal reflux disease, liver disease and diseases of the large intestine are important pathologies that impact on prognosis and also impair quality of life. Diagnosis and management of gastrointestinal manifestations will be discussed in this review. Since optimisation of CF therapy is associated with a significantly longer life-span of CF patients nowadays, also gastrointestinal malignancies, which are more common in CF than in the non-CF population need to be considered. Furthermore, novel evidence on the role of the gut microbiome in CF is emerging. The introduction of cystic fibrosis transmembrane conductance regulator (CFTR) protein modulators gives hope for symptom alleviation and even cure of gastrointestinal manifestations of CF. (c) 2020 The Author(s). Published by Elsevier Ltd. This is an open access article under the CC BY-NC-ND license (http://creativecommons.org/licenses/by-nc-nd/4.0/).

Automated summary

Cystic fibrosis primarily affects the lungs, but it also has significant gastrointestinal manifestations that impact diagnosis, prognosis, and quality of life. This review discusses the various gastrointestinal issues associated with CF, including pancreatic insufficiency, gastroesophageal reflux disease, liver disease, and diseases of the large intestine. The optimization of CF therapy has led to longer life spans for CF patients, but it is important to consider the increased risk of gastrointestinal malignancies in this population. Additionally, emerging evidence suggests that the gut microbiome plays a role in CF. The introduction of CFTR protein modulators brings hope for alleviating symptoms and potentially curing gastrointestinal manifestations of CF.