Prognosis and disease severity in cystic fibrosis (CF) are linked to declining lung function. To characterise lung function by the number of adults in countries with different levels of Gross National Income (GNI), data from the European Cystic Fibrosis Society Patient Registry were utilised.

Annual data including age, forced expiratory volume in 1 s (FEV₁), anthropometry, genotype, respiratory cultures and CF-related diabetes (CFRD) were retrieved between 2011 and 2021. All countries were stratified into GNI per capita to reflect differences within Europe.

A consistent improvement in FEV₁ % pred and survival was observed among the 47 621 people with CF (pwCF), including subjects with chronic Pseudomonas aeruginosa infection, CFRD and/or undernutrition. Mean values of FEV₁ % pred changed from 85% to 94.2% for children and from 63.6% to 74.7% for adults. FEV₁ % pred further increased among those carrying the F508del mutation in 2021, when elexacaftor/tezacaftor/ivacaftor was available. The number of adult pwCF increased from 13 312 in 2011 to 21 168 in 2021, showing a 60% increase. PwCF living in European lower income countries did not demonstrate a significant annual increase in FEV₁ % pred or in the number of adults.

This pan-European analysis demonstrates a consistent improvement in FEV₁ % pred, number of adult pwCF and survival over the last decade only in European higher and middle income countries. Urgent action is needed in the lower income countries where such improvement was not observed. The notable improvement observed in pwCF carrying the F508del mutation emphasises the need to develop treatments for all CF mutations.

囊性纤维化（CF）的预后和疾病严重程度与肺功能下降有关。为了通过不同国民总收入 (GNI) 水平的国家的成年人数量来表征肺功能，利用了欧洲囊性纤维化协会患者登记处的数据。

检索 2011 年至 2021 年间的年度数据，包括年龄、1 秒用力呼气量 (FEV ₁ )、人体测量学、基因型、呼吸培养和 CF 相关糖尿病 (CFRD)。所有国家均按人均国民总收入分层，以反映欧洲内部的差异。

在 47 621 名 CF (pwCF) 患者中观察到FEV ₁ % pred 和生存率持续改善，其中包括患有慢性铜绿假单胞菌感染、CFRD 和/或营养不良的受试者。_{儿童的 FEV 1} % pred平均值从 85% 变为 94.2%，成人从 63.6% 变为 74.7%。2021 年，当 elexacaftor/tezacaftor/ivacaftor 上市时，携带 F508del 突变的患者的FEV _{1 % pred 进一步增加。}成人pwCF人数从2011年的13 312人增加到2021年的21 168人，增长了60%。生活在欧洲低收入国家的 PwCF 并未表现出 FEV ₁ % pred 或成年人数量的显着年度增长。

这项泛欧分析表明，过去十年中，只有欧洲中高收入国家的FEV _{1 % pred、成人 pwCF 数量和生存率持续改善。}尚未观察到这种改善的低收入国家需要采取紧急行动。在携带 F508del 突变的 pwCF 中观察到的显着改善强调需要开发针对所有 CF 突变的治疗方法。