PT  - JOURNAL ARTICLE
AU  - Vincent Cottin
AU  - David Hansell
AU  - Katerina Antoniou
AU  - Arjun Nair
AU  - Mark Atwood
AU  - Nicola Sverzellati
AU  - Gerry Oster
AU  - Derek Weycker
AU  - Harold R. Collard
AU  - Athol Wells
TI  - The decline in lung function is related to both emphysema and fibrosis extent in patients with idiopathic pulmonary fibrosis (IPF)/ combined pulmonary fibrosis and emphysema (CPFE)
DP  - 2014 Sep 01
TA  - European Respiratory Journal
PG  - P747
VI  - 44
IP  - Suppl 58
4099  - http://erj.ersjournals.com/content/44/Suppl_58/P747.short
4100  - http://erj.ersjournals.com/content/44/Suppl_58/P747.full
SO  - Eur Respir J2014 Sep 01; 44
AB  - Background: Concurrent emphysema may be associated with spurious preservation of FVC in CPFE. Serial FVC measurement used to monitor progression in IPF might fail to capture disease progression in CPFE.Aim: To evaluate the change in FVC% predicted over time in patients with IPF and CPFE in relation to the extent of emphysema and fibrosis on HRCT.Methods: The extent of fibrosis and emphysema on baseline HRCT scans was quantified by visual estimation in 309 patients participating in a placebo-controlled clinical trial of interferon γ-1b with evaluable HRCT scans. The diagnosis of IPF was made using ATS/ERS 2000 criteria, with surgical lung biopsy in 58.3% of patients.Results: The mean age was 66.0±8.1 years, including 225 males (72.8%) with 68.0% former and 5.2% current smokers. At baseline, FVC% predicted was 73.2±13.5% and DLco was 46.9±8.6%. Emphysema was present in 121 patients (39%). The extent of emphysema was negatively related with the extent of fibrosis (r = -0.277, p < 0.001 ; and test of linear trend, p=0.01). The 48-week decrease in FVC% predicted was correlated with the extent of fibrosis (r = 0.249; p < 0.001), and inversely correlated with the extent of emphysema (r= -0.115; p=0.044). Patients with less fibrosing disease and those with a higher extent of emphysema showed less decline of FVC% predicted, but no less decline in DLco and no greater increase in CPI score.Conclusions: In patients with IPF and associated emphysema (CPFE), change in FVC may not be a sensitive measure of disease progression. These findings have implications for both patient management and clinical trial design.