Pirfenidone improves survival in IPF: results from a real-life study

BMC Pulm Med. 2018 Nov 23;18(1):177. doi: 10.1186/s12890-018-0736-z.


Background: Pirfenidone is an antifibrotic compound approved for the treatment of idiopathic pulmonary fibrosis (IPF). We present our real-world experience in terms of Pirfenidone's effect on mortality and adverse events profile outside the restrictions of a clinical trial.

Methods: This is a retrospective observational intention to treat study of 82 consecutive IPF patients (UHH cohort).

Results: We observed a high 3-years survival rate of 73% without excluding patients who discontinued treatment for different reasons. The survival was compared to the survival of an IPF cohort from a tertiary referral center (RBH cohort). After exclusion of severe cases (DLco< 30%), in unadjusted analysis, the survival in the UHH cohort was better than in the RBH cohort (HR:0.32, 95% CI: 0.19-0.53, p < 0.0001). After adjustment for age, gender and FVC, the survival remained higher in the UHH cohort (HR:0.28, 95% CI: 0.16-0.48, p < 0.0001). We observed a similar safety profile compared to previously published data and a lower rate of drug discontinuation due to photosensitivity reactions.

Conclusion: Pirfenidone provides a survival benefit in a real-life IPF cohort compared to previously used medications. Counselling patients and proactively managing possible adverse effects can reduce the necessity to discontinue pirfenidone.

Keywords: IPF; Pirfenidone; Real-life; Survival.

Publication types

  • Observational Study

MeSH terms

  • Aged
  • Aged, 80 and over
  • Anti-Inflammatory Agents, Non-Steroidal / therapeutic use*
  • Disease Progression
  • Female
  • Greece
  • Humans
  • Idiopathic Pulmonary Fibrosis / drug therapy*
  • Idiopathic Pulmonary Fibrosis / mortality*
  • Male
  • Middle Aged
  • Pyridones / therapeutic use*
  • Retrospective Studies
  • Survival Analysis
  • Time Factors
  • Treatment Outcome
  • Vital Capacity


  • Anti-Inflammatory Agents, Non-Steroidal
  • Pyridones
  • pirfenidone