title: Pirfenidone for Idiopathic Pulmonary Fibrosis: A Systematic Review and Meta-Analysis.
abstract: Idiopathic pulmonary fibrosis (IPF) is a progressive disease with poor prognosis. In the last decades pirfenidone an anti-inflammatory and anti-fibrotic agent has shown benefit in inhibit collagen production and has also demonstrated benefit in decline progression in IPF in physiological outcomes as Forced vital capacity (FVC), in clinical outcomes such as progression free survival (PFS) and a benefit in mortality but no in clinically relevant outcomes as exacerbations or worsening of IPF.
  
  
  METHODS
  We conducted a systematic review to evaluate the effectiveness of physiological and clinical outcomes of pirfenidone compared to placebo in IPF. We performed a search with no language restriction. Two researchers performed literature search, quality assessment, data extraction and analysis. And was performed a summary of findings table following the GRADE approach.
  
  
  RESULTS
  We included 5 RCTs (Randomized controlled trials) in analysis. The meta-analysis resulted in a decrease in all cause-mortality (RR 0.52 IC 0.32-0.88) and IPF related mortality (RR 0.32 IC 0.14-0.75); other outcomes evaluated were worsening of IPF (RR 0.64 IC 0.50-0.83) and acute exacerbation (RR: 0.72 IC 0.30-1.66 respectively). Also there was a decrease in the risk of progression (RR of PFS: 0.82 IC 0.73–0.92) compared to placebo. Conclusions: We observed significant differences in physiologic and clinically relevant outcomes such as reduction in all-cause mortality, IPF related mortality, worsening of IPF and improvement of PFS. So pirfenidone treatment should be considered not only for its benefits in pulmonary function tests but also by its clinically relevant outcomes [corrected].
authors: Department of Respiratory diseases, Faculty of Medicine, Pontificia Universidad Católica de Chile, Santiago, Chile.
authors: Carlos
authors: C
authors: Aravena
authors: Department of Internal Medicine, Faculty of Medicine, Pontificia Universidad Católica de Chile, Santiago, Chile; Evidence Based Health Care Program, Faculty of Medicine, Pontificia Universidad Católica de Chile, Santiago, Chile.
authors: Gonzalo
authors: G
authors: Labarca
authors: Department of Respiratory diseases, Faculty of Medicine, Pontificia Universidad Católica de Chile, Santiago, Chile.
authors: Carmen
authors: C
authors: Venegas
authors: Department of Internal Medicine, Faculty of Medicine, Pontificia Universidad Católica de Chile, Santiago, Chile.
authors: Alex
authors: A
authors: Arenas
authors: Department of Internal Medicine, Faculty of Medicine, Pontificia Universidad Católica de Chile, Santiago, Chile; Evidence Based Health Care Program, Faculty of Medicine, Pontificia Universidad Católica de Chile, Santiago, Chile; Epistemonikos foundation, Santiago, Chile.
authors: Gabriel
authors: G
authors: Rada
mesh_terms: D000894:Anti-Inflammatory Agents, Non-Steroidal / Q000627:therapeutic use*; D006801:Humans; D054990:Idiopathic Pulmonary Fibrosis / Q000188:drug therapy*; D011728:Pyridones / Q000627:therapeutic use*
chemical_list: D000894:Anti-Inflammatory Agents, Non-Steroidal; D011728:Pyridones; C093844:pirfenidone