TY - JOUR T1 - Risk factors for forced expiratory volume in 1 s decline in European patients with cystic fibrosis: data from the European Cystic Fibrosis Society Patient Registry JF - ERJ Open Research JO - erjor DO - 10.1183/23120541.00449-2022 VL - 9 IS - 3 SP - 00449-2022 AU - Elpis Hatziagorou AU - Steffen Fieuws AU - Annalisa Orenti AU - Lutz Naehrlich AU - Uros Krivec AU - Meir Mei-Zahav AU - Andreas Jung AU - Kris De Boeck AU - on behalf of the ECFSPR Collaborative Group AU - ECFSPR Collaborative Group AU - Pfleger Andreas AU - Géraldine Daneau AU - Lammertijn Elise AU - Petrova Guergana AU - Pavel Drevinek AU - Milan Macek, Jr AU - Hanne Vebert Olesen AU - Pierre-Régis Burgel AU - Lydie Lemonnier-Videau AU - Andrea Párniczky AU - Godfrey Fletcher AU - Rita Padoan AU - Anna Zolin AU - Elina Aleksejeva AU - Kestutis Malakauskas AU - Vincent Gulmans AU - Stojka Fustik AU - Ivana Arnaudova Danevskai AU - Oxana Turcu AU - Luísa Pereira AU - Liviu Pop AU - Elena Kondratyeva AU - Milan Rodić AU - Hana Kayserová AU - M. Dolores Pastor-Vivero AU - Isabelle de Monestrol AU - Anders Lindblad AU - Deniz Dogru AU - Halyna Makukh AU - Siobhán B. Carr AU - Rebecca Cosgriff Y1 - 2023/05/01 UR - http://openres.ersjournals.com/content/9/3/00449-2022.abstract N2 - Aim To examine the trajectory of forced expiratory volume in 1 s (FEV1) using data from the European Cystic Fibrosis Society patient registry (ECFPR) collected from 2008 to 2016, i.e. the era before highly effective modulator therapy (HEMT). We evaluated risk factors for FEV1 decline.Methods The study population included patients with a confirmed diagnosis of cystic fibrosis recorded in the ECFPR (2008–2016). The evolution of FEV1 % predicted (%FEV1) with age, and the yearly change in %FEV1 were evaluated. Risk factors considered were cystic fibrosis transmembrane conductance regulator (­CFTR) mutation class, gender, age at diagnosis, neonatal screening, meconium ileus, sweat chloride concentration at diagnosis and country's income level.Results We used 199 604 FEV1 recordings from 38 734 patients. The fastest decline was seen during puberty and in patients diagnosed before the age of 10 years. Males had a higher %FEV1, but a higher yearly %FEV1 loss between the ages of 15 and 25 years. We showed stabilisation and even improvement in %FEV1 over age in adults with a class III CFTR mutation, but a steady decline in patients homozygous for F508del or with both mutations of classes I/II. A faster decline in %FEV1 was found in patients from low-income countries compared to a similar %FEV1 evolution in patients from middle- and high-income countries.Conclusions These longitudinal FEV1 data reflect the reality of cystic fibrosis across Europe in the era pre-HEMT, and can serve as baseline for comparison with the post-HEMT era. The similar evolution in middle- and high-income countries underlines opportunities for low-income countries.Longitudinal data in people with CF show a different FEV1 evolution in patients with class III mutation, the only group on effective modulators. Similar FEV1 evolution in middle- and high-income countries underlines opportunities for low-income countries. https://bit.ly/3YgIOIo ER -