Antifibrotic treatment efficacy in progressive pulmonary fibrosis: single center experience

<bold>Introduction:</bold> In cases with pulmonary fibrosis due to interstitial lung diseases (ILD), the presence of at least two of the three criteria defined as worsening of respiratory symptoms, functional deterioration and radiological progression within the last year is considered as progressive pulmonary fibrosis (PPF). In our study, the effectiveness of antifibrotics in cases with PPF other than idiopathic pulmonary fibrosis was investigated. <bold>Method:</bold> Demographics, clinical features, 6-minute walking distance (6MWD), spirometry and diffusion capacity (DLCO) measurements of the patients with PPF who used antifibrotics for at least 6 months were recorded. Functional parameters at the sixth month and 1st year of treatment were compared with baseline data. <bold>Results:</bold> Totally 105 cases diagnosed with PPF were included. Thirty-nine cases (37%) had systemic sclerosis, 22 (16%) had hypersensitivity pneumonitis, 12 (11%) had rheumatoid arthritis, and 32 (30%) had other ILD. Majority of the cases (n=91, 86.7%) were using nintedanib while 14 cases using pirfenidone. In the pre-antifibrotic treatment evaluation, the mean FVC was 65.37±17.758%, DLCO was 47.15±14.796%, 6MWD was 403.89±89.148 meters. When compared with baseline values, there was no significant loss in FVC and DLCO at the sixth month and first year. However, there was a significant increase in 6MWD (p<0.001 at 6 months, p<0.001 at 1 year). Except for 12 cases who desaturated during 6-minute walk test before treatment, no new cases with desaturation were detected during follow-up. <bold>Conclusion:</bold> Our results suggest that rapid functional loss can be prevented with antifibrotic treatment in cases with PPF.