Efficacy and safety of inhaled N-acetylcysteine in idiopathic pulmonary fibrosis: A prospective, single-arm study.
BACKGROUND
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 352.4mg 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), 2.27L and %FVC, 76.2%. The mean change in FVC during 26 weeks prior to the inhaled N-acetylcysteine therapy was -170mL, a significant decrease (p=0.019). The mean change in FVC during 26 weeks after the initiation of inhaled N-acetylcysteine therapy was -70mL (p=0.06). When the patients were classified into two groups according to the degree of decline in FVC (≥100mL vs. <100mL) 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.).
Department of Respiratory Medicine, Saitama Red Cross Hospital, 8-3-33 Kami-ochiai, Chuo-ku, Saitama 338-8553, Japan. Electronic address: b980013@yahoo.co.jp.
Ryo
R
Okuda
Department of Respiratory Medicine, Saitama Red Cross Hospital, 8-3-33 Kami-ochiai, Chuo-ku, Saitama 338-8553, Japan. Electronic address: himatsushima@sunny.ocn.ne.jp.
Hidekazu
H
Matsushima
Department of Respiratory Medicine, Saitama Red Cross Hospital, 8-3-33 Kami-ochiai, Chuo-ku, Saitama 338-8553, Japan. Electronic address: obatomohiro@hotmail.co.jp.
Tomohiro
T
Oba
Department of Respiratory Medicine, Saitama Red Cross Hospital, 8-3-33 Kami-ochiai, Chuo-ku, Saitama 338-8553, Japan. Electronic address: blackberry_wine1218@yahoo.co.jp.
Rie
R
Kawabe
Department of Respiratory Medicine, Saitama Red Cross Hospital, 8-3-33 Kami-ochiai, Chuo-ku, Saitama 338-8553, Japan. Electronic address: mizu_mina@hotmail.com.
Minako
M
Matsubayashi
Department of Respiratory Medicine, Saitama Red Cross Hospital, 8-3-33 Kami-ochiai, Chuo-ku, Saitama 338-8553, Japan. Electronic address: skyfield1021@gmail.com.
Masako
M
Amano
Department of Respiratory Medicine, Saitama Red Cross Hospital, 8-3-33 Kami-ochiai, Chuo-ku, Saitama 338-8553, Japan. Electronic address: tn1230bungo@yahoo.co.jp.
Tomotaka
T
Nishizawa
Department of Respiratory Medicine, Saitama Red Cross Hospital, 8-3-33 Kami-ochiai, Chuo-ku, Saitama 338-8553, Japan. Electronic address: hondakojiro78@gmail.com.
Koujiro
K
Honda
D000111:Acetylcysteine / Q000008:administration & dosage*; D000280:Administration, Inhalation; D000368:Aged; D005260:Female; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000188:drug therapy* / Q000503:physiopathology*; D008297:Male; D012129:Respiratory Function Tests; D012720:Severity of Illness Index; D013997:Time Factors; D016896:Treatment Outcome; D014797:Vital Capacity
D000111:Acetylcysteine
Forced vital capacity; Idiopathic pulmonary fibrosis; Inhalation; Krebs von den Lungen-6; N-acetylcysteine