Efficacy and safety of inhaled N-acetylcysteine in idiopathic pulmonary fibrosis: A prospective, single-arm study

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• 作者：Ryo Okuda ; ^{b980013@yahoo.co.jp" class="auth_mail" title="E-mail the corresponding author} ; Hidekazu Matsushima ^{himatsushima@sunny.ocn.ne.jp" class="auth_mail" title="E-mail the corresponding author} ; Tomohiro Oba ^{obatomohiro@hotmail.co.jp" class="auth_mail" title="E-mail the corresponding author} ; Rie Kawabe ^{blackberry_wine1218@yahoo.co.jp" class="auth_mail" title="E-mail the corresponding author} ; Minako Matsubayashi ^{mizu_mina@hotmail.com" class="auth_mail" title="E-mail the corresponding author} ; Masako Amano ^{skyfield1021@gmail.com" class="auth_mail" title="E-mail the corresponding author} ; Tomotaka Nishizawa ^{tn1230bungo@yahoo.co.jp" class="auth_mail" title="E-mail the corresponding author} ; Koujiro Honda ^{hondakojiro78@gmail.com" class="auth_mail" title="E-mail the corresponding author}
• 关键词：IPF ; idiopathic pulmonary fibrosis ; FVC ; forced vital capacity ; VC ; vital capacity ; DLco ; diffusion capacity of the lung for carbon monoxide ; UIP ; usual interstitial pneumonia ; KL-6 ; Krebs von den Lungen-6 ; SP-D ; surfactant protein-D
• 刊名：Respiratory Investigation
• 出版年：2016
• 出版时间：May 2016
• 年：2016
• 卷：54
• 期：3
• 页码：156-161
• 全文大小：422 K

文摘

Idiopathic pulmonary fibrosis (IPF) is a progressive lung disease with few treatment options. The efficacy of N-acetylcysteine in patients with IPF remains controversial. The aim of this research was to investigate the efficacy of inhaled N-acetylcysteine.

Methods

This study was designed as a single-center, single-arm, prospective clinical trial. Each patient who had IPF received 352.4 mg of inhaled N-acetylcysteine twice daily.

Results

In total, 28 patients were enrolled. The mean values of the respiratory function parameters at the initiation of therapy were as follows: forced vital capacity (FVC), 2.27 L and %FVC, 76.2%. The mean change in FVC during 26 weeks prior to the inhaled N-acetylcysteine therapy was −170 mL, a significant decrease (p=0.019). The mean change in FVC during 26 weeks after the initiation of inhaled N-acetylcysteine therapy was −70 mL (p=0.06). When the patients were classified into two groups according to the degree of decline in FVC (≥100 mL vs. <100 mL) during the 26-week period prior to the initiation of therapy, inhaled N-acetylcysteine showed a greater efficacy in attenuating FVC decline in the ≥100-mL group than in the <100-mL group.

Conclusions

Inhaled N-acetylcysteine therapy was effective in patients with mild-to-moderate IPF and was more beneficial in patients who had greater declines in FVC before the initiation of therapy. (UMIN title: Efficacy and safety of inhaled N-acetylcysteine in idiopathic pulmonary fibrosis, UMIN000016706, 2015/03/04.)