Effectiveness of pirfenidone for idiopathic pulmonary fibrosis associated with pleuroparenchymal fibroelastosis-like lesions and nonspecific interstitial pneumonia.

Susumu SakamotoHiroshige ShimizuTakuma IsshikiYasuhiko NakamuraAtsuko KurosakiKazutoshi IsobeSakae Homma

Published in: The clinical respiratory journal (2020)

Pirfenidone decreased the decline in FVC in patients with UIP+PPFELL and UIP+NSIP, as well as in those with typical IPF. However, outcomes were worse for patients with UIP+PPFELL.

Keyphrases

idiopathic pulmonary fibrosis
interstitial lung disease
randomized controlled trial
systematic review
type diabetes
intensive care unit
adipose tissue
skeletal muscle
acute respiratory distress syndrome
mechanical ventilation