Risk factors for FEV1 decline in European patients with CF: data from the European Cystic Fibrosis Society Patient Registry (ECFSPR)

Aim To examine the trajectory of forced expiratory volume in 1 s (FEV1) using data from European Cystic Fibrosis (CF) Society Patient Registry collected 2008 to 2016, hence era before highly effective modulator therapy (HEMT). We evaluated risk factors for FEV1 decline. Methods The study population included patients with a confirmed diagnosis CF recorded ECFPR (2008–2016). evolution FEV1% predicted age (%FEV1), and yearly change %FEV1 were evaluated. Risk considered CFTR mutation class, gender, at diagnosis, neonatal screening, meconium ileus, sweat chloride concentration country's income level. Results used 199,604 recordings 38,734 patients. fastest decline was seen during puberty diagnosed ten years. Males had higher but loss between ages 15 25. showed stabilization even improvement over adults class III steady homozygous F508del or both mutations classes I/II. A faster found 'low-income' countries compared similar middle- high-income countries. Conclusions These longitudinal reflect reality across Europe pre-HEMT can serve as baseline comparison post-HEMT era. underlines opportunities low-income