RT Journal Article
SR Electronic
T1 Effect of pirfenidone on IPF progression
JF European Respiratory Journal
JO Eur Respir J
FD European Respiratory Society
SP PA2091
DO 10.1183/13993003.congress-2016.PA2091
VO 48
IS suppl 60
A1 Diana Radicella
A1 Ginevra Del Giudice
A1 Assunta Micco
A1 Mariano Mazza
A1 Mario Del Donno
YR 2016
UL http://erj.ersjournals.com/content/48/suppl_60/PA2091.abstract
AB Background: IPF is a chronic,progressive and lethal interstitial lung disease with poor prognosis.Although pirfenidone is tolerable and safe while reducing lung function decline, few studies document the trend of progression in UIP patients before starting treatment with pirfenidone.Objectives:The purpose of this study is to evaluate the progression of IPF pre and during pirfenidone treatment.Methods:We recruited 20 patients with mild-moderate IPF diagnosis (during the years 2011-2016) according to guidelines. All patients were studied with pulmonary function tests including spirometry, Body Plethysmography , DLCO and 6-minute walk test during one year pretreatment with pirfenidone (T-12), 6 month post introducing treatment (T6) and 1 year post-treatment (T+12).Results: We observed an initial decline in DLCO mean[mean value 54,1% pred (DS 13,26)],FVC [meanvalue 83% pred (DS 19,7)] SpO2 [mean value 95.5% (DS 2.39)] parameters at T-12. The trend of disease progression in mild-moderate IPF remained stable at T6 [FVC mean 77,1 (DS 21,46)] and T+12 [FVC% 76,75 (DS 19,75)] post pirfenidone introduction.However, patients with worse initial data, show a greater decline in DLCO, in 6MWT distance and lowest SpO2% than patients with better initial data.In addition, five patients with four years of treatment, show FVC and DLCO stability.Conclusions: Our data show that pirfenidone determines the stability of pulmonary function parameters in mild-moderate IPF.