Pirfenidone, nintedanib and N-acetylcysteine for the treatment of idiopathic pulmonary fibrosis: A systematic review and meta-analysis

2016 ◽  
Vol 40 ◽  
pp. 95-103 ◽  
Author(s):  
Paola Rogliani ◽  
Luigino Calzetta ◽  
Francesco Cavalli ◽  
Maria Gabriella Matera ◽  
Mario Cazzola
Keyword(s):  
Systematic Review ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Meta Analysis

Prognostic role of Krebs von den Lungen-6 (KL-6) measurement in idiopathic pulmonary fibrosis: a systematic review and meta-analysis

2021 ◽  
Vol 0 (0) ◽  
Author(s):  
Elena Aloisio ◽  
Federica Braga ◽  
Chiara Puricelli ◽  
Mauro Panteghini
Keyword(s):  
Systematic Review ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Binary Data ◽  
Meta Analysis ◽  
Mortality Prediction ◽  
Asian Populations ◽  
Increased Risk ◽  
Using Data ◽  
Patient Prognosis

Abstract Objectives Idiopathic pulmonary fibrosis (IPF) is a progressive interstitial disease with limited therapeutic options. The measurement of Krebs von den Lungen-6 (KL-6) glycoprotein has been proposed for evaluating the risk of IPF progression and predicting patient prognosis, but the robustness of available evidence is unclear. Methods We searched Medline and Embase databases for peer-reviewed literature from inception to April 2020. Original articles investigating KL-6 as prognostic marker for IPF were retrieved. Considered outcomes were the risk of developing acute exacerbation (AE) and patient survival. Meta-analysis of selected studies was conducted, and quantitative data were uniformed as odds ratio (OR) or hazard ratio (HR) estimates, with corresponding 95% confidence intervals (CI). Results Twenty-six studies were included in the systematic review and 14 were finally meta-analysed. For AE development, the pooled OR (seven studies) for KL-6 was 2.72 (CI 1.22–6.06; p=0.015). However, a high degree of heterogeneity (I2=85.6%) was found among selected studies. Using data from three studies reporting binary data, a pooled sensitivity of 72% (CI 60–82%) and a specificity of 60% (CI 52–68%) were found for KL-6 measurement in detecting insurgence of AE in IPF patients. Pooled HR (seven studies) for mortality prediction was 1.009 (CI 0.983–1.036; p=0.505). Conclusions Although our meta-analysis suggested that IPF patients with increased KL-6 concentrations had a significant increased risk of developing AE, the detection power of the evaluated biomarker is limited. Furthermore, no relationship between biomarker concentrations and mortality was found. Caution is also needed when extending obtained results to non-Asian populations.

scholarly journals Efficacy and safety of pirfenidone in the treatment of idiopathic pulmonary fibrosis patients: a systematic review and meta-analysis of randomised controlled trials

BMJ Open ◽  
2021 ◽  
Vol 11 (12) ◽  
pp. e050004
Author(s):  
Wenjuan Wu ◽  
Lingxiao Qiu ◽  
Jizhen Wu ◽  
Xueya Liu ◽  
Guojun Zhang
Keyword(s):  
Systematic Review ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Adverse Events ◽  
Acute Exacerbation ◽  
Randomised Controlled Trials ◽  
Meta Analysis ◽  
Controlled Trials ◽  
Efficacy And Safety ◽  
Randomised Controlled

ObjectivesIdiopathic pulmonary fibrosis (IPF) has been defined as a distinctive type of chronic fibrotic disease, characterised by a progressive decline in lung function and a common histological pattern of interstitial pneumonia. To analyse the efficacy and safety of pirfenidone in the treatment of IPF, a systematic review and meta-analysis was performed.DesignThis is a meta-analysis study.ParticipantsPatients were diagnosed as IPF.InterventionsUse of pirfenidone.Primary and secondary outcomeProgression-free survival (PFS), acute exacerbation and worsening of IPF and Impact on adverse events.MeasuresThe inverse variance method for the random-effects model was used to summarise the dichotomous outcomes, risk ratios and 95% CIs.ResultsA total of 9 randomised controlled trials with 1011 participants receiving pirfenidone and 912 controls receiving placebo were summarised. The pooled result suggested a statistically significant difference inall-cause mortality after pirfenidone use, with a summarised relative ratio of 0.51 (p<0.01). Longer PFS was observed in patients receiving pirfenidone compared with those who were given placebo (p<0.01). The IPF groups presented a high incidence of adverse events with a pooled relative ratio of 3.89 (p<0.01).ConclusionsPirfenidone can provide survival benefit for patients with IPF. Pirfenidone treatment was also associated with a longer PFS, a lower incidence of acute exacerbation and worsening of IPF.

Systematic Review and Meta-analysis of Pirfenidone, Nintedanib, and Pamrevlumab for the Treatment of Idiopathic Pulmonary Fibrosis

2020 ◽  
pp. 106002802096445
Author(s):  
Enrica Di Martino ◽  
Alessio Provenzani ◽  
Patrizio Vitulo ◽  
Piera Polidori
Keyword(s):  
Systematic Review ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Meta Analysis ◽  
Random Effect ◽  
Random Effect Model ◽  
World Health ◽  
Effect Model ◽  
All Cause Mortality ◽  
Positive Effect

Background: The comparative efficacy of pirfenidone, nintedanib, and pamrevlumab in slowing the rate of forced vital capacity (FVC) decline and mortality in patients with idiopathic pulmonary fibrosis (IPF) is unknown. Objective: To perform a systematic review and meta-analysis (MA) of these drugs for IPF. Methods: We searched CENTRAL, PubMed, EMBASE, ClincalTrials.gov, and the World Health Organization’s registry databases up to March 2020. Phase II/III randomized controlled trials in adults with IPF were eligible. The random-effect model was implemented calculating the effect size and respective 95% CI as Cohen’s d for change from baseline FVC (in percentage predicted and liters) and odds ratio (OR) for 10% reduction in FVC and all-cause mortality (ACM). Results: Six studies were included in the MA. For change from baseline in percentage predicted FVC, the MA indicated that the 3 drugs were more effective than placebo (pirfenidone: d=3.30%, 95% CI=2.15-4.45; nintedanib: d=3.15%, 95% CI=2.35-3.95; pamrevlumab: d=4.30%, 95% CI=0.45-8.15). These results are superimposable to those relating to change from baseline FVC in liters (pirfenidone: d=0.09L, 95% CI=0.04-0.14; nintedanib: d=0.13L, 95% CI=0.10-0.16; pamrevlumab: d=0.20L, 95% CI=0.05-0.35). Each drug had a positive effect on 10% reduction in FVC (pirfenidone: OR=0.57, 95% CI=0.45-0.74; nintedanib: OR=0.66, 95% CI=0.51-0.85; pamrevlumab: OR=0.24, 95% CI=0.08-0.73), but only pirfenidone showed an effect on ACM (OR=0.50; 95% CI=0.31-0.83). Conclusion and Relevance: This MA provided encouraging results on pamrevlumab efficacy in slowing the decline in FVC compared with pirfenidone and nintedanib. Actually, in phase 3, it could become a potential IPF treatment.

scholarly journals Systematic review and network meta-analysis of approved medicines for the treatment of idiopathic pulmonary fibrosis

2019 ◽  
Vol 8 (1) ◽  
pp. 55-61 ◽  
Author(s):  
Aristeidis Skandamis ◽  
Chara Kani ◽  
Sophia L. Markantonis ◽  
Kyriakos Souliotis
Keyword(s):  
Systematic Review ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Meta Analysis

scholarly journals Prognostic factors for acute exacerbation of idiopathic pulmonary fibrosis: protocol for a systematic review and meta-analysis

BMJ Open ◽  
2019 ◽  
Vol 9 (5) ◽  
pp. e028226
Author(s):  
Hiroyuki Kamiya ◽  
Ogee Mer Panlaqui
Keyword(s):  
Systematic Review ◽  
Prognostic Factors ◽  
Prognostic Factor ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Acute Exacerbation ◽  
Case Reports ◽  
Meta Analysis ◽  
Scientific Journal ◽  
Grade Method

IntroductionIdiopathic pulmonary fibrosis (IPF) is a form of chronic fibrosing interstitial pneumonia with unknown disease aetiology. Acute exacerbation (AE) of IPF is an accelerated disease progression beyond its expected course. A 30-day mortality of AE of IPF is 40%. While death may occur, there is much variation in the clinical progression of this condition. Previous attempts have been made to investigate various possible prognostic factors for AE of IPF; however, they have yet to be confirmed. The aim of this systematic review is to clarify these prognostic factors.Methods and analysisIn this review, AE of IPF is the condition of interest, which has been defined according to previously established diagnostic criteria. The primary outcomes of interest include short-term all-cause mortality and pulmonary-cause mortality. The secondary outcomes of interest include long-term mortality and hospital separation for the disease. Primary studies investigating prognostic factors for AE of IPF are eligible for inclusion in this review. All study types are permitted except case reports. Two reviewers will search electronic databases, such as Medline and EMBASE, from 2002 to the 1 April 2019 and extract data independently. Risk of bias in individual studies will be assessed using the Quality in Prognostic Studies tool. Meta-analysis will be conducted for univariate data if at least three studies report the effect of a specific prognostic factor using similar statistical methods. Multivariate results will be reported qualitatively. Subgroup analysis and sensitivity analysis will be considered with the aim of generalising findings to the clinical settings and drawing more robust conclusions. The Grades of Recommendation, Assessment, Development and Evaluation (GRADE) method will be applied to evaluate the quality of evidence for each prognostic factor.Ethics and disseminationEthical approval will not be required. Results will be reported in a peer-reviewed scientific journal.PROSPERO registration numberCRD42018106172

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

PLoS ONE ◽  
2015 ◽  
Vol 10 (10) ◽  
pp. e0140288 ◽  
Author(s):  
Carlos Aravena ◽  
Gonzalo Labarca ◽  
Carmen Venegas ◽  
Alex Arenas ◽  
Gabriel Rada
Keyword(s):  
Systematic Review ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Meta Analysis

Sildenafil for idiopathic pulmonary fibrosis: A systematic review and meta-analysis

2021 ◽  
Author(s):  
Tyler Pitre ◽  
Muhammad Faran Khalid ◽  
Sonya Cui ◽  
Melanie C. Zhang ◽  
Renata Husnudinov ◽  
...  
Keyword(s):  
Systematic Review ◽  
Idiopathic Pulmonary Fibrosis ◽  
Pulmonary Fibrosis ◽  
Meta Analysis ◽  
Rate Of Change ◽  
Point Estimate ◽  
Control Group ◽  
Randomized Controlled Studies ◽  
Significant Difference ◽  
Acute Exacerbations

Abstract Background: Patients with idiopathic pulmonary fibrosis have a poor overall prognosis and there are few evidence based drug therapies that reduce mortality. Objective: This systematic review and meta-analysis aims to assess whether sildenafil reduces mortality, reduces disease progression and the adverse side effects associated with it. Methods: In this review, randomized controlled studies (RCTs) were retrieved from MEDLINE, Cochrane, and EMBASE. The primary outcome was mortality. The secondary outcomes included change in FVC, acute exacerbations and hospitalizations and adverse drug effects leading to discontinuation. We used an inverse variance random effects meta-analysis method to calculate pooled odds ratio (OR) and standardized mean difference (SMD). Results: A total of 4 studies were included. Sildenafil probably reduces mortality when compared to placebo or to standard care, [OR 0.63 (0.38,1.03), I2=0%]. Pooled results showed sildenafil does not alter the rate of change of FVC [SMD 0.02 (-0.14,0.18)], or DLCO [SMR -0.01 (-0.18,0.17)], I2=0]. Pooled results showed sildenafil may not reduce the number of hospitalizations or acute exacerbations, [OR 1.06 (0.67,1.67)], I2= 0]. There was no significant difference in drug discontinuation due to adverse effects when comparing sildenafil to the control group, [OR 0.79 (0.56, 1.11)], I2=0]. Conclusion: Sildenafil probably reduces all-cause mortality in IPF patients. More studies need to be done in order to confirm the magnitude and reliability of the point estimate.

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