AUTHOR=Tzouvelekis Argyrios , Karampitsakos Theodoros , Ntolios Paschalis , Tzilas Vasilios , Bouros Evangelos , Markozannes Evangelos , Malliou Ioanna , Anagnostopoulos Aris , Granitsas Andreas , Steiropoulos Paschalis , Dimakou Katerina , Chrysikos Serafeim , Koulouris Nikolaos , Bouros Demosthenes TITLE=Longitudinal “Real-World” Outcomes of Pirfenidone in Idiopathic Pulmonary Fibrosis in Greece JOURNAL=Frontiers in Medicine VOLUME=4 YEAR=2017 URL=https://www.frontiersin.org/journals/medicine/articles/10.3389/fmed.2017.00213 DOI=10.3389/fmed.2017.00213 ISSN=2296-858X ABSTRACT=Background

Pirfenidone is an antifibrotic compound able to slow down disease progression in patients with idiopathic pulmonary fibrosis (IPF).

Objective

To investigate the safety and efficacy of pirfenidone in patients with IPF in a real-life setting.

Methods

This was a multicenter, retrospective, real-life, observational study for patients with IPF receiving pirfenidone.

Results

We identified 92 patients with IPF receiving pirfenidone. Eighty patients (70 males and 10 females, mean age ± SD: 68.1 + 7.5, mean %FVC ± SD = 74.9 ± 17.2, mean %DLCO ± SD = 48.1 ± 16.9) were included in the analysis. Skin-related (25%) and gastrointestinal (17.5%) adverse events were the most common and led to drug discontinuation in 22.5% of cases. The majority (87%) of patients experienced side effects during the first 6 months of treatment. At 36 months, changes in %FVC and %DLCO were −9.25 ± 16.34 and −9.26 ± 15.26, respectively. At 6, 12, and 24 months after treatment initiation (n = 80, 60, and 26), 18, 15, and 5 patients (22.5, 25, and 19.2%) experienced significant (>10%) and 11, 3, and 3 patients (13.8, 5, and 11.5%) experienced marginal (5–10%) %FVC improvement; and 13, 6, and 1 patient (16.2, 10, and 3.9%) experienced marginal (−5 to −10%) and 20, 21, and 8 patients (25, 35, and 30.8%) experienced significant decline (<−10%) in %FVCpred. Median survival was 851 days, and 41 patients died during the study period.

Conclusion

Pirfenidone demonstrated an acceptable safety and therapeutic profile in patients with IPF on a longitudinal basis. Prospective observational registries are urgently needed to provide a real-world view of outcomes of pirfenidone in clinical practice.