Pulmonary function test in patients with idiopathic pulmonary fibrosis: data from the National Registry for Moscow and Moscow region

Abstract

The purpose of the work was to evaluate the frequency and severity of lung functional disorders, the relationship between respiratory function (RF) and clinical symptoms in patients with idiopathic pulmonary fibrosis (IPF). Materials and Methods . The National Registry registered IPF patients ( n = 127; 90 (71%) men, 37 (29%) women; average age 69.5 ± 8.5 years), by March 2019 lived in Moscow and Moscow region; at the time of inclusion in the register the duration of the disease was 3.3 ± 3.1 years (22 (17%) < 1 year). The diagnosis of IPF is based on the clinical picture of the disease, the results of high-resolution computed tomography and/or histological examination of the material obtained by surgical lung biopsy. The data of the complex RF examination (spirometry, Body plethysmography, lung diffusion capacity by carbon monoxide – DL CO ), 6-minute walking test (6-MWT) and dyspnea evaluation according to the modified dyspnea scale (Modified Medical Research Council – mMRC) at the moment of inclusion in the register are analyzed. Results . Average value of forced vital capacity (FVC) was 78 ± 22% debt, ratio of forced expiratory volume for 1 s/FVC – 83 ± 9%, total lung capacity (TLC) – 77 ± 17% debt, DL CO – 47 ± 18% debt. DL CO , TLC, FVC were reduced in 97.5, 63 and 59% of patients respectively. In patients with a disease duration of less than 1 year, DL CO was significantly higher than that of less than 1 year. The median distance covered by the 6-MWT was 317.5 m, dyspnea according to the mMRC scale – 2 points. Statistically significant correlations between RF values and shortness of breath, tolerance to physical activity have been revealed. Conclusion . In patients with IPF, included in the National Register, restrictive disorders of pulmonary ventilation were detected in the majority of cases, reduced DL CO – in 97.5%. Statistically significant higher DL CO values are noted for a disease duration of less than 1 year.