Elexacaftor-Tezacaftor-Ivacaftor (ETI) has substantially improved lung function and life expectancy for people with cystic fibrosis (pwCF). Emerging evidence suggests that overweight, obesity and dyslipidaemia are now developing in pwCF prescribed ETI. This scoping review, conducted adhering to PRISMA guidelines, aimed to provide an overview of the research currently available that investigated body mass index (BMI), body composition and serum lipid profiles in adults with CF treated with ETI. Peer-reviewed articles and conference abstracts were identified through database searches and conference proceedings. Studies reporting BMI, body composition (fat mass, fat-free mass) and serum lipid profiles in adults with CF following ETI initiation were included. Data were extracted and synthesised descriptively. Of 126 studies (60 peer-reviewed, 66 conference abstracts), 90% reported increased BMI after ETI initiation, with 61% indicating this was statistically significant. Fat and fat-free mass rose significantly in 5 of 8 and 4 of 7 studies, respectively. In the 18 studies (11 peer-reviewed publications, 7 conference abstracts) reporting serum lipids, significant increases were observed; for total cholesterol (72% of studies), LDL-C (80%), and HDL-C (56%), with increased triglycerides (31%), non-HDL cholesterol and HDL: Total Cholesterol ratio (22% for both) less frequently seen. Current evidence suggests that BMI, weight, and serum lipids increase in adults with CF following ETI initiation, with some studies also reporting increased fat- and fat-free mass. The majority of measurements remain within the recommended range. These findings highlight the importance of ongoing monitoring of nutritional status and cardiometabolic health in this population. Prospective research to better characterise long-term cardiometabolic health in pwCF is required.
Walsh et al. (Sun,) studied this question.