P175 Single centre experience of the real-life impact of pirfenidone on lung function in patients with idiopathic pulmonary fibrosis

IntroductionIdiopathic pulmonary fibrosis (IPF) is a progressively destructive lung disease that culminates in respiratory failure and death. Trials have demonstrated that treatment of IPF patients with Pirfenidone reduces %FVC decline, improves progression-free survival and significantly reduces th...

Full description

Saved in:
Bibliographic Details
Published in:Thorax 2016-12, Vol.71 (Suppl 3), p.A179-A179
Main Authors: Rathnapala, A, Fries, A, West, Y, Ho, LP, Hoyles, RK
Format: Article
Language:English
Citations: Items that cite this one
Online Access:Get full text
Tags: Add Tag
No Tags, Be the first to tag this record!
Description
Summary:IntroductionIdiopathic pulmonary fibrosis (IPF) is a progressively destructive lung disease that culminates in respiratory failure and death. Trials have demonstrated that treatment of IPF patients with Pirfenidone reduces %FVC decline, improves progression-free survival and significantly reduces the risk of all-cause mortality at 1 year. Our anecdotal experience is that a small proportion of patients show improvement of %FVC with treatment.ObjectivesTo assess the proportion of patients in an ILD specialist centre that improve, stabilise or decline in their %FVCs on Pirfenidone treatment.MethodsIn this retrospective study patients with IPF diagnosed according to the ATS/ERS guidelines at the ILD MDT, who were commenced and continued on Pirfenidone for >6 months were included. Data was derived from the clinical records of the Oxford IPF clinic.Results100 patient records were analysed and 31 were excluded (n = 15 10%) decline in %FVC.After one year of Pirfenidone (n = 44 patients), 3 (6.8%) showed significant and 5 (11.4%) showed marginal improvement, 18 (40.9%) showed stability, 11 (25%) showed mild and 7 (15.9%) showed significant decline of %FVC.After 2 years of treatment (n = 15 patients), 1 (6.7%) showed significant and 3 (20%) showed mild improvement, 4 (26.7%) showed stability, 3 (20%) showed mild and 4 (26.7%) showed significant decline of the %FVC.Among 8 patients who had improvement in %FVC at one year, 6 were males, 6 had definite IPF, median age 77 years (68 – 84) and the median FVC was 73.5% predicted (66 – 79).ConclusionsReal-life use of Pirfenidone shows clear slowing of decline in the %FVC, whereas a clinically significant subset show improvement in FVC. Potentially the beneficial effect is lost after 22–24 months, although small numbers limit this analysis.Abstract P175 Figure 1Pre and Post- pirfenidone treatment
ISSN:0040-6376
1468-3296
DOI:10.1136/thoraxjnl-2016-209333.318