RT期刊文章电子T1 Pirfenidone pl SRus inhaled N-acetylcysteine for idiopathic pulmonary fibrosis: a randomised trial JF European Respiratory Journal JO Eur Respir J FD European Respiratory Society SP 2000348 DO 10.1183/13993003.00348-2020 VO 57 IS 1 A1 Sakamoto, Susumu A1 Kataoka, Kensuke A1 Kondoh, Yasuhiro A1 Kato, Motoyasu A1 Okamoto, Masaki A1 Mukae, Hiroshi A1 Bando, Masashi A1 Suda, Takafumi A1 Yatera, Kazuhiro A1 Tanino, Yoshinori A1 Kishaba, Tomoo A1 Hattori, Noboru A1 Taguchi, Yoshio A1 Saito, Takefumi A1 Nishioka, Yasuhiko A1 Kuwano, Kazuyoshi A1 Kishi, Kazuma A1 Inase, Naohiko A1 Sasaki, Shinichi A1 Takizawa, Hajime A1 Johkoh, Takeshi A1 Sakai, Fumikazu A1 Homma, Sakae YR 2021 UL //www.qdcxjkg.com/content/57/1/2000348.abstract AB Background A randomised controlled trial in Japan showed that inhaled N-acetylcysteine monotherapy stabilised serial decline in forced vital capacity (FVC) in some patients with early idiopathic pulmonary fibrosis (IPF). However, the efficacy and tolerability of combination therapy with an antifibrotic agent and inhaled N-acetylcysteine are unknown.Methods This 48-week, randomised, open-label, multicentre phase 3 trial compared the efficacy and tolerability of combination therapy with pirfenidone plus inhaled N-acetylcysteine 352.4 mg twice daily with the results for pirfenidone alone in patients with IPF. The primary end-point was annual rate of decline in FVC. Exploratory efficacy measurements included serial change in diffusing capacity of the lung for carbon monoxide (DLCO) and 6-min walk distance (6MWD), progression-free survival (PFS), incidence of acute exacerbation, and tolerability.Results 81 patients were randomly assigned in a 1:1 ratio to receive pirfenidone plus inhaled N-acetylcysteine (n=41) or pirfenidone (n=40). The 48-week rate of change in FVC was −300 mL and −123 mL, respectively (difference −178 mL, 95% CI −324–−31 mL; p=0.018). Serial change in DLCO, 6MWD, PFS and incidence of acute exacerbation did not significantly differ between the two groups. The incidence of adverse events (n=19 (55.9%) for pirfenidone plus N-acetylcysteine; n=18 (50%) for pirfenidone alone) was similar between groups.Conclusions Combination treatment with inhaled N-acetylcysteine and pirfenidone is likely to result in worse outcomes for IPF.We compared the efficacy of pirfenidone plus inhaled N-acetylcysteine with results for pirfenidone alone for IPF. Combination treatment with inhaled N-acetylcysteine and pirfenidone is likely to result in worse outcomes for IPF. https://bit.ly/3eWEbvW