Abstract
Introduction
In this retrospective italian study that included all major national interstitial lung diseases centers we observed the impact of Pirfenidone therapy (PT) in IPF patients (pts) evaluating the degree of disease progression before and after starting PT.
Methods
We retrospectively analyzed 128 pts with mild, moderate and severe IPF comparing functional decline before and after the introduction of PT. Eligible pts had at least 6 months of follow-up before starting PT and at least 6 months after starting PT.
Results
At baseline (first Pirfenidone prescription) mean percentage predicted forced vital capacity (FVC) was 0,75 (0,35-1,43), mean percentage predicted diffuse lung capacity (DLCO) was 0,47 (0,17-1,20). Dividing population in stages according to GAP index: 48 (37.5%) were stage I, 64 (50%) stage II, 8 (6.3%) stage III, 8 missing.
In the whole population PT reduced the slop of decline of FVC (p=0.049), not of DLCO (p=0.244) in comparison to pre-treatment period.
Splitting population in two sub-groups: FVC >= 0.75, FVC <0.75, decline in FVC (p=0.002) and GAP index (p=0.028) were significantly lower in the second group. No changes were detected for the decline of DLCO (p=0,686).
Sub-group analysis was also performed using GAP index at baseline (I vs II/III). Once again a decrease in decline in functional parameters in the more severe functional group (GAP Index and FVC, the latter not significant) was detected.
Conclusions
In this real life national experience PT reduced the slope of decline of FVC, not of DLCO. PT could be effective also for pts with moderate-severe disease and this finding needs further investigations.
- © 2014 ERS
