TDNet Discover

Objective: The purpose of this systematic review is to determine if the use of elexacaftor, tezacaftor and ivacaftor (ETI) therapy is associated with improvement in morbidity and surrogate clinical data for people with cystic fibrosis (pwCF) with at least one F508del CF mutation. Background: PsA is a leading cause of morbidity and mortality in pwCF. Current treatment guidelines include usage of inhaled antibiotics, steroids, and non-steroidal anti-inflammatory drugs (NSAIDs). Advancements in cystic fibrosis transmembrane conductance regulator (CFTR) modulators are a relatively new therapy that has promising initial results in the management of CF. Methods: Systematic review of articles published between 2017-2023 in PubMed, Medline and Google Scholar that assessed ETI therapy for pwCF. Morbidity and surrogate clinical data analyzed include: ppFEV1, SCC, BMI, Immune & Inflammatory Response, CT Scans, Endoscopy, Microbial Diversity, and Adverse Effects of Pharmacotherapy. Results: This review included: 9 prospective cohort studies, 3 retrospective cohort studies, and 1 prospective/retrospective cohort study. The results indicated a statistically significant decrease in PsA colonization, improvements in immune and inflammatory responses, and improvements in clinical markers such as percent predicted forced expiratory volume in 1 second (ppFEV1), body mass index (BMI), and sweat chloride concentrations (SCC). Conclusions: ETI therapy clears PsA colonization and improves lung function, immune response, inflammatory response, and quality of life in pwCF.