Frontiers in Medicine (Nov 2017)

Longitudinal “Real-World” Outcomes of Pirfenidone in Idiopathic Pulmonary Fibrosis in Greece

  • Argyrios Tzouvelekis,
  • Argyrios Tzouvelekis,
  • Theodoros Karampitsakos,
  • Paschalis Ntolios,
  • Vasilios Tzilas,
  • Evangelos Bouros,
  • Evangelos Markozannes,
  • Ioanna Malliou,
  • Aris Anagnostopoulos,
  • Andreas Granitsas,
  • Paschalis Steiropoulos,
  • Katerina Dimakou,
  • Serafeim Chrysikos,
  • Nikolaos Koulouris,
  • Demosthenes Bouros

DOI
https://doi.org/10.3389/fmed.2017.00213
Journal volume & issue
Vol. 4

Abstract

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BackgroundPirfenidone is an antifibrotic compound able to slow down disease progression in patients with idiopathic pulmonary fibrosis (IPF).ObjectiveTo investigate the safety and efficacy of pirfenidone in patients with IPF in a real-life setting.MethodsThis was a multicenter, retrospective, real-life, observational study for patients with IPF receiving pirfenidone.ResultsWe 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.ConclusionPirfenidone 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.

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