Pirfenidone for Idiopathic Pulmonary Fibrosis: A Systematic Review and Meta-Analysis

<div><p>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 progression free survival (PFS) (RR 0.83 IC 0.74–0.92) 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 and exacerbation of IPF and PFS. So pirfenidone treatment should be considered not only for its benefits in pulmonary function tests but also by its clinically relevant outcomes.</p></div

Risk of bias summary: review authors' judgment’s about each risk of bias item for each included study.

<p>Risk of bias summary: review authors' judgment’s about each risk of bias item for each included study.</p

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<p>Adverse events.</p

Comparison 4.

<p>Acute exacerbation.</p

Comparison 10.

<p>Skin related adverse events.</p

Comparison 7.

<p>Change on 6-minutes walked test (6MWT) distance.</p

Summary of finding form Pirfenidone for idiopathic pulmonary fibrosis.

<p>1: Non primary outcome from RCTs, 2: High heterogeneity; 6MWT: Six minutes walk test; RCT: Randomized controlled trial; RR: Risk ratio; CI: confidence interval.</p><p>Summary of finding form Pirfenidone for idiopathic pulmonary fibrosis.</p

Characteristics of included studies in this systematic review.

<p>Characteristics of included studies in this systematic review.</p

PRISMA flow diagram.

<p>PRISMA flow diagram.</p

Summary of adverse events.

<p>Right: Pirfenidone group; left: placebo group N.D: No data.</p><p>Summary of adverse events.</p