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吸入性N-乙酰半胱氨酸对特发性肺纤维化患者的长期疗效

Long-term efficacy of inhaled N-acetylcysteine in patients with idiopathic pulmonary fibrosis.

作者信息

Bando Masashi, Hosono Tatsuya, Mato Naoko, Nakaya Takakiyo, Yamasawa Hideaki, Ohno Shoji, Sugiyama Yukihiko

机构信息

Division of Pulmonary Medicine, Department of Medicine, Jichi Medical University, Tochigi.

出版信息

Intern Med. 2010;49(21):2289-96. doi: 10.2169/internalmedicine.49.4011. Epub 2010 Nov 1.

DOI:10.2169/internalmedicine.49.4011
PMID:21048362
Abstract

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.

CONCLUSION

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.

摘要

背景

自1994年起,我科就开始采用吸入N - 乙酰半胱氨酸(NAC)治疗特发性肺纤维化(IPF)等间质性肺炎。本研究旨在探讨长期NAC吸入单一疗法治疗IPF的临床疗效及安全性。

方法

1994年至2008年间,我科对34例经外科肺活检确诊为IPF的患者中的23例进行了NAC吸入治疗。其中14例患者持续治疗一年或更长时间。对这14例患者以及11例未接受治疗的患者,检查了其临床病程、预后、肺功能(%FVC、%DLco和%TLC)以及间质性肺炎血清标志物(KL - 6和SP - D)的变化。

结果

两组的生存曲线无显著差异。接受NAC吸入治疗的14例患者中有4例(28.6%)出现急性加重。与NAC吸入治疗开始前的结果相比,治疗组患者一年后的Δ%FVC和Δ%DLco分别为 - 4.7%和 - 2.9%,两年后分别为 - 4.0%和 - 5.8%。未治疗组患者一年后的Δ%FVC和Δ %DLco分别为 - 3.5%和 + 5.3%,两年后分别为 + 0.2%和 + 1.0%。

结论

由于本研究是单机构的开放性病例对照研究,且病例数量不多,其在评估NAC吸入单一疗法疗效方面的应用有限。此外,还应研究NAC吸入联合类固醇、免疫抑制剂和新型抗纤维化药物的作用。

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