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

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

Author information

1
Respiratory Medicine Department, University Hospital of Heraklion, Crete, Greece.
2
Interstitial Lung Disease Unit, Royal Brompton Hospital, London, UK.
3
Radiology Department, University Hospital of Heraklion, Crete, Greece.
4
Respiratory Medicine Department, University Hospital of Heraklion, Crete, Greece. kantoniou@med.uoc.gr.

Abstract

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

PMID:
30470213
PMCID:
PMC6251092
DOI:
10.1186/s12890-018-0736-z
[Indexed for MEDLINE]
Free PMC Article

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