期刊
THERAPEUTIC ADVANCES IN RESPIRATORY DISEASE
卷 6, 期 2, 页码 107-114出版社
SAGE PUBLICATIONS LTD
DOI: 10.1177/1753465812436663
关键词
idiopathic pulmonary fibrosis; antifibrotic; pirfenidone; vital capacity; prognostic factor; evidence-based (EB) guideline; phenostage
资金
- Diffuse Lung Diseases Research Grant from the Ministry of Health, Labour and Welfare, Japan
Pirfenidone is the first antifibrotic agent to be approved for the treatment of pulmonary fibrosis. Idiopathic pulmonary fibrosis (IPF) is one of the idiopathic interstitial pneumonias with the worst prognoses; approximately half of patients die within 3-5 years, and the need for an effective treatment has been unmet until recently. The etiology of IPF is still unknown and its pathogenesis is poorly understood. Anti-inflammatory drugs, such as corticosteroids and some immunosuppressants, have been empirically used to treat IPF, although they have not been objectively proven to be effective by large-scale randomized, controlled trials. Pirfenidone is an agent that can inhibit the decline of forced vital capacity (FVC)/vital capacity (VC) and that thereby can be hoped to decrease the mortality rate. The number of clinical trials of pirfenidone completed, ongoing, or planned is growing, and the present status of pirfenidone as treatment for IPF is summarized in this review.
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