PT  - JOURNAL ARTICLE
AU  - H. Taniguchi
AU  - M. Ebina
AU  - Y. Kondoh
AU  - T. Ogura
AU  - A. Azuma
AU  - M. Suga
AU  - Y. Taguchi
AU  - H. Takahashi
AU  - K. Nakata
AU  - A. Sato
AU  - M. Takeuchi
AU  - G. Raghu
AU  - S. Kudoh
AU  - T. Nukiwa
AU  - the Pirfenidone Clinical Study Group in Japan
TI  - Pirfenidone in idiopathic pulmonary fibrosis
AID  - 10.1183/09031936.00005209
DP  - 2010 Apr 01
TA  - European Respiratory Journal
PG  - 821--829
VI  - 35
IP  - 4
4099  - http://erj.ersjournals.com/content/35/4/821.short
4100  - http://erj.ersjournals.com/content/35/4/821.full
SO  - Eur Respir J2010 Apr 01; 35
AB  - Idiopathic pulmonary fibrosis (IPF) is a progressive lung disease without proven effective therapy. A multicentre, double-blind, placebo-controlled, randomised phase III clinical trial was conducted in Japanese patients with well-defined IPF to determine the efficacy and safety of pirfenidone, a novel antifibrotic oral agent, over 52 weeks. Of 275 patients randomised (high-dose, 1,800 mg·day−1; low-dose, 1,200 mg·day−1; or placebo groups in the ratio 2:1:2), 267 patients were evaluated for the efficacy of pirfenidone. Prior to unblinding, the primary end-point was revised; the change in vital capacity (VC) was assessed at week 52. Secondary end-points included the progression-free survival (PFS) time. Significant differences were observed in VC decline (primary end-point) between the placebo group (-0.16 L) and the high-dose group (-0.09 L) (p = 0.0416); differences between the two groups (p = 0.0280) were also observed in the PFS (the secondary end-point). Although photosensitivity, a well-established side-effect of pirfenidone, was the major adverse event in this study, it was mild in severity in most of the patients. Pirfenidone was relatively well tolerated in patients with IPF. Treatment with pirfenidone may decrease the rate of decline in VC and may increase the PFS time over 52 weeks. Additional studies are needed to confirm these findings.