Idiopathic pulmonary fibrosis: pathogenesis and management

G Sgalla, B Iovene, M Calvello, M Ori, F Varone… - Respiratory …, 2018 - Springer
Background Idiopathic pulmonary fibrosis (IPF) is a chronic, progressive disease
characterized by the aberrant accumulation of fibrotic tissue in the lungs parenchyma …

Interstitial lung disease in connective tissue disorders

A Fischer, R Du Bois - The lancet, 2012 - thelancet.com
Some of the most pressing challenges associated with interstitial lung disease (ILD) are how
best to define, diagnose, and treat connective tissue disease-associated ILD (CTD-ILD) …

Pirfenidone for idiopathic pulmonary fibrosis: analysis of pooled data from three multinational phase 3 trials

PW Noble, C Albera, WZ Bradford… - European …, 2016 - Eur Respiratory Soc
Pirfenidone is an antifibrotic agent that has been evaluated in three multinational phase 3
trials in patients with idiopathic pulmonary fibrosis (IPF). We analysed pooled data from the …

Pirfenidone in patients with idiopathic pulmonary fibrosis (CAPACITY): two randomised trials

PW Noble, C Albera, WZ Bradford, U Costabel… - The Lancet, 2011 - thelancet.com
Background Idiopathic pulmonary fibrosis is a progressive and fatal lung disease with
inevitable loss of lung function. The CAPACITY programme (studies 004 and 006) was …

Antifibrotic activities of pirfenidone in animal models

CJ Schaefer, DW Ruhrmund, L Pan… - European …, 2011 - Eur Respiratory Soc
Pirfenidone is an orally active small molecule that has recently been evaluated in large
clinical trials for the treatment of idiopathic pulmonary fibrosis, a fatal disease in which the …

Drug treatment of idiopathic pulmonary fibrosis: systematic review and network meta-analysis

WJ Canestaro, SH Forrester, G Raghu, L Ho… - Chest, 2016 - Elsevier
Background Idiopathic pulmonary fibrosis (IPF) is a form of chronic progressive fibrosing
interstitial lung disease of unknown origin. Recently, nintedanib and pirfenidone …

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

P Rogliani, L Calzetta, F Cavalli, MG Matera… - Pulmonary …, 2016 - Elsevier
Background The prevalence of idiopathic pulmonary fibrosis (IPF) is increasing every year.
Pirfenidone and nintedanib were approved for treatment of IPF in 2014, but they received …

Exploring efficacy and safety of oral Pirfenidone for progressive, non-IPF lung fibrosis (RELIEF)-a randomized, double-blind, placebo-controlled, parallel group, multi …

J Behr, P Neuser, A Prasse, M Kreuter, K Rabe… - BMC pulmonary …, 2017 - Springer
Background Pirfenidone is currently approved in the EU for the treatment of mild to moderate
idiopathic pulmonary fibrosis (IPF) and offers a beneficial risk-benefit profile. However, there …

Investigating the possible mechanisms of pirfenidone to be targeted as a promising anti-inflammatory, anti-fibrotic, anti-oxidant, anti-apoptotic, anti-tumor, and/or anti …

SA Antar, MA Saleh, AA Al-Karmalawy - Life Sciences, 2022 - Elsevier
Pirfenidone (PFD) is a non-peptide synthetic chemical that inhibits the production of
transforming growth factor-beta 1 (TGF-β1), tumor necrosis factor-alpha (TNF-α), platelet …

Treating idiopathic pulmonary fibrosis with the addition of co-trimoxazole: a randomised controlled trial

L Shulgina, AP Cahn, ER Chilvers, H Parfrey, AB Clark… - Thorax, 2013 - thorax.bmj.com
Background Idiopathic pulmonary fibrosis (IPF) is a fatal condition with limited treatment
options. However, in a previous small study, co-trimoxazole was found to be beneficial …