OBJECTIVES: Cystic fibrosis (CF) is a rare, progressive disorder affecting the lungs, pancreas, and other organs, and has a significant burden on patients' functioning and quality of life (QoL). We aimed to identify comprehensive evidence of the impact of CF on the health-related quality of life (HRQoL) in five key European countries.

METHODS: We systematically searched Embase^® and MEDLINE^® databases via Embase.com to identify English-language articles published from 2013–2023 reporting QoL data for CF in children and adults in the UK, France, Germany, Italy and Spain.

RESULTS: Among the 1,365 records screened, 22 studies were included reporting data for the UK (14), Germany (4), France (1), Spain (1), Italy (1) and all five countries (1). EQ-5D and Cystic Fibrosis Questionnaire-Revised (CFQ-R) were the most widely used QoL scales in all the studies. Mean EQ-5D utility values for participants were: 0.667 (France), 0.783 (Germany), 0.820 (Italy), 0.870 (Spain) and 0.640 (UK). EQ-5D and CFQ-R data showed that the worse the pulmonary exacerbation, the poorer the HRQoL. In addition to lung function, transplant status, age, body mass index, or having CF-related diabetes, B. cepacia complex or an implantable vascular access device affected many HRQoL domains. CF transmembrane conductance regulator modulators have shown improvements in disease progression and QoL. In a study conducted in Germany, elexacaftor/tezacaftor/ivacaftor combination improved CFQ-R respiratory domain score by 27.9 (IQR 5.6 to 47.2; p < 0.001).

CONCLUSIONS: Although several factors are associated with QoL in children and adults with CF, pulmonary exacerbations and disease duration have the largest impact on HRQoL. Appropriate treatment can help in improving QoL. Further research is required to investigate the impact of other associated factors.