PT  - JOURNAL ARTICLE
AU  - Franziska Ihle
AU  - Tobias Veith
AU  - Zimmermann Gregor
AU  - Wypior Gabriela
AU  - Behr Juergen
AU  - Neurohr Claus
TI  - Pirfenidone therapy for patients with progressive idiopathic pulmonary fibrosis
DP  - 2014 Sep 01
TA  - European Respiratory Journal
PG  - P776
VI  - 44
IP  - Suppl 58
4099  - http://erj.ersjournals.com/content/44/Suppl_58/P776.short
4100  - http://erj.ersjournals.com/content/44/Suppl_58/P776.full
SO  - Eur Respir J2014 Sep 01; 44
AB  - Background: The aim of this observational study was to evaluate pirfenidone in patients with progressive idiopathic pulmonary fibrosis (IPF).Methods:Patients were evaluated by repetitive pulmonary function testing (PFT) 6 months prior and minimum 6 months during pirfenidone therapy. A decrease of the Forced Vital Capacity (FVC) >5%pred. and/or a diffusing capacity for carbon monoxide (DLCO) >10%pred. was defined as IPF progression (treatment non-responder). Student's T Test was used to assessdifferences in PFT values.Results: 31 consecutive patients (age 61±9.0years; 22male [71%]) received pirfenidone. Before the start of pirfenidone FVC%pred., DLCO and oxygen partial pressure were 59.9%pred., 33.5%pred. and 58.1 mmHg, respectively. The mean loss of FVC before the administration period did not differ significantly between responder (n=21, 68%) and non-responder (n=10; 32%; -2.18±5.78 % pred. vs. -1.73 ± 5.92%pred., p=0.843). However, at the end of the observation the FVC loss of responders was significantly less compared to non-responders (1.52±4.62%pred. vs -7.41±2.39%pred., p<0.001). Moreover, there was a significant decline of the FVC within the non-responder group (-1.73±5.92%pred. vs -7.41±2.39%pred., p=0.021). The intra-individual course of the responder group tend to increase without reaching statistical significance (-2.18±5.78%pred. vs 1.52±4.62%pred., p=0.085).Conclusions: Our data suggest that the majority of progressive IPF patients respond to pirfenidone therapywithin the study period.