Pirfenidone as anti-fibrosis drug developed in recent years demonstrated the potential anti- fibrotic effect, but so far there were no domestic studies about pirfenidone's efficacy and safety evaluation in china. The aim of this study was to evaluate the efficacy and safety of pirfenidone in patients with idiopathic pulmonary fibrosis (IPF) through the observation of a large sample of clinical cases.

The aim of this study was to evaluate the efficacy and safety of pirfenidone in patients with idiopathic pulmonary fibrosis (IPF) through the observation of a large sample of clinical cases. During the observation, study visits will occur at the end of 12w, 24w, 36w, 48w.all participants will be required to check the various efficacy and safety indicators.

Inclusion Criteria:

1. Written informed consent signed;
2. Age ≤75 years;
3. Clinically or multidisciplinary diagnosed idiopathic pulmonary fibrosis(see 2011 guidance );
4. Resting state PaO2≥50mg, FVC%≥45% normal predicted value and DLCO≥30% normal predicted value.

Exclusion Criteria:

1. Allergic to pirfenidone;
2. Patients with serious Significant pulmonary infection need anti-infection treatment;
3. Patients who has taken interferon, penicillamine or other agents for the treatment of IPF;
4. Patients who has taken prednisone(≥50mg) or other glucocorticoid in the past 1 month;
5. Patients who has taken immunosuppressants in the past 1 month;
6. Patients who has taken amiodarone which may cause pulmonary fibrosis in the past 3 months;
7. Patients with malignant tumor in the past 5 years;
8. Participated in other clinical trials in the past 3 months;
9. Patients with serious heart disease(NYHA class Ⅲ-Ⅳ), liver disease(ALT or AST 2 times above the upper level of normal value range), kidney disease(Cr above the upper level of normal value range);
10. Pregnant or lactating women;
11. The investigator assessed as inappropriate to participate in this clinical trial.