Dynamics of lung function in patients with cystic fibrosis living in the Volga Federal District in 2017 and 2023

Cystic fibrosis (CF) is a severe hereditary disease that leads to decreased lung function. Objective. To assess the dynamics of lung function changes in CF patients living in the Volga Federal District (VFD) regions based on data from the 2017 and 2023 registries. Material and methods. Spirometry data from 375 patients from the 2017 CF patient registry and 451 patients from the 2023 registry were analyzed. Results. The median FEV1 data in VFD patients was 76.8% of the predicted value in 2017 and significantly increased to 95.3% of the predicted value in 2018. in 2023 (p<0.001). In 2017, FEV1 values in patients with CF in the Volga Federal District were not significantly different from those in the Russian Federation (75.5% of the predicted value), while in 2023, they were significantly higher than in patients in other regions of Russia (81.1% of the predicted value), p<0.001. FEV1 values increased significantly in patients from the Nizhny Novgorod Region, Orenburg Region, Perm Krai, the Republic of Tatarstan, and the Chuvash Republic. The median FVC in patients with CF from the Volga Federal District in 2017 was 85.1% of the predicted value and significantly increased to 94.0% of the predicted value in 2023 (p<0.01). In 2017, the FVC indicators in patients with CF in the Volga Federal District did not differ significantly from those in patients in the Russian Federation (86.0% of the predicted value), and in 2023 they were significantly higher than in patients in other regions of Russia (91.0% of the predicted value), p=0.037. The FVC indicators in 2023, compared with 2017, significantly increased in patients in the Nizhny Novgorod Region (p<0.001), Orenburg Region (p<0.001), Perm Krai (p=0.021), the Republic of Tatarstan (p=0.043), and the Chuvash Republic (p=0.003). In patients in the Samara Region, the indicators remained stable (p=0.331). Conclusions. FEV1 and FVC values in CF patients in both the Volga Federal District and the Russian Federation increased significantly in 2023 compared to 2017. Patient data from Volga Federal District regions where specialized CF centers were established were more informative, and patients had higher lung function values.