Pirfenidone therapy for patients with progressive idiopathic pulmonary fibrosis
F. Ihle, T. Veith, Z. Gregor, W. Gabriela, B. Juergen, N. Claus (Munich, Germany)
Source: International Congress 2014 – ILDs 3
Session: ILDs 3
Session type: Thematic Poster Session
Number: 776
Disease area: Interstitial lung diseases
Abstract 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.
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F. Ihle, T. Veith, Z. Gregor, W. Gabriela, B. Juergen, N. Claus (Munich, Germany). Pirfenidone therapy for patients with progressive idiopathic pulmonary fibrosis. Eur Respir J 2014; 44: Suppl. 58, 776
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