Long-term efficacy of inhaled N-acetylcysteine in patients with idiopathic pulmonary fibrosis.
BACKGROUND
Inhalation of N-acetylcysteine (NAC) has been carried out in our department since 1994 for treating interstitial pneumonia such as idiopathic pulmonary fibrosis (IPF). In this study, the clinical efficacy and safety of long-term NAC inhalation monotherapy for IPF was investigated.


METHODS
NAC inhalation was carried out in 23 of 34 cases diagnosed as IPF by surgical lung biopsy in our department between 1994 and 2008. The treatment was continued for one year or longer in 14 cases. In these 14 cases and in 11 cases without treatment, the clinical courses, prognosis, lung function (%FVC, %DLco, and %TLC), and changes in serum markers for interstitial pneumonia (KL-6 and SP-D) were examined.


RESULTS
There were no significant differences in survival curves between the two groups. Acute exacerbation was observed in 4 of 14 cases (28.6%) receiving NAC inhalation. Compared with the results just before the beginning of NAC inhalation, Δ%FVC and Δ%DLco in the treated cases were -4.7% and -2.9% one year later and -4.0% and -5.8% two years later, respectively. In cases without treatment, Δ%FVC and Δ%DLco were -3.5% and +5.3% one year later and +0.2% and +1.0% two years later, respectively.


CONCLUSIONS
Since this study is an open case-control study in a single institute and the number of cases is not large, its use in evaluating the efficacy of NAC inhalation monotherapy is limited. In addition, the role of NAC inhalation in combination with a steroid, an immunosuppressive agent, and a new anti-fibrosis drug should also be investigated.
Division of Pulmonary Medicine, Department of Medicine, Jichi Medical University, Tochigi. bando034@jichi.ac.jp
Masashi
M
Bando
Tatsuya
T
Hosono
Naoko
N
Mato
Takakiyo
T
Nakaya
Hideaki
H
Yamasawa
Shoji
S
Ohno
Yukihiko
Y
Sugiyama
D000111:Acetylcysteine / Q000008:administration & dosage*; D000280:Administration, Inhalation; D000368:Aged; D016022:Case-Control Studies; D005260:Female; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000175:diagnosis / Q000188:drug therapy* / Q000401:mortality; D008297:Male; D008875:Middle Aged; D012129:Respiratory Function Tests / Q000639:trends; D012189:Retrospective Studies; D013997:Time Factors; D016896:Treatment Outcome
D000111:Acetylcysteine