UTILIZATION OF A PROMISING NOVEL TREATMENT PROTOCOL FOR AN ACUTE EXACERBATION OF IDIOPATHIC PULMONARY FIBROSIS ULTIMATELY INEFFECTIVE

CHEST Journal ◽  
2018 ◽  
Vol 154 (4) ◽  
pp. 461A
Author(s):  
VINAY GOSWAMY ◽  
IGNACIO PORTALES CASTILLO ◽  
SUMON ROY ◽  
HARINDER SINGH ◽  
LAURIE LERNER
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 ◽  
...  

2019 ◽  
Vol 53 (2) ◽  
pp. 1801699 ◽  
Author(s):  
Coralynn Sack ◽  
Ganesh Raghu

Idiopathic pulmonary fibrosis (IPF) is a progressive fibrotic lung disease of unknown origin that is associated with high morbidity and mortality. In this perspective, we briefly review the current understanding of the pathophysiology of IPF and the importance of environmental triggers as a precipitant of disease. We discuss occult intrinsic and extrinsic environmental factors that affect the lung microenvironment and may contribute to the development and progression of disease. The clinical implications of this framework need to be further elucidated, because prompt identification and elimination of occult exposures may represent a novel treatment modality.


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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