scholarly journals Acute Exacerbation of Idiopathic Pulmonary Fibrosis: Outcome and Prognostic Factors

Respiration ◽  
2012 ◽  
Vol 83 (1) ◽  
pp. 28-35 ◽  
Author(s):  
Virginie Simon-Blancal ◽  
Olivia Freynet ◽  
Hilario Nunes ◽  
Diane Bouvry ◽  
Nicolas Naggara ◽  
...  
BMJ Open ◽  
2019 ◽  
Vol 9 (5) ◽  
pp. e028226
Author(s):  
Hiroyuki Kamiya ◽  
Ogee Mer Panlaqui

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


2018 ◽  
Vol 57 (5) ◽  
pp. 655-661 ◽  
Author(s):  
Kenichiro Atsumi ◽  
Yoshinobu Saito ◽  
Naoyuki Kuse ◽  
Kenichi Kobayashi ◽  
Toru Tanaka ◽  
...  

2018 ◽  
Vol 10 (9) ◽  
pp. 5346-5358 ◽  
Author(s):  
Takefumi Nikaido ◽  
Yoshinori Tanino ◽  
Xintao Wang ◽  
Yuki Sato ◽  
Ryuichi Togawa ◽  
...  

2017 ◽  
Vol 83 (5) ◽  
Author(s):  
Gaetano Rea ◽  
Giuseppe Fiorentino ◽  
Antonio Corcione ◽  
Maurizia Lanza ◽  
Francesco Perna ◽  
...  

BMJ Open ◽  
2021 ◽  
Vol 11 (12) ◽  
pp. e050004
Author(s):  
Wenjuan Wu ◽  
Lingxiao Qiu ◽  
Jizhen Wu ◽  
Xueya Liu ◽  
Guojun Zhang

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.


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