Summary
Background: FIBRONET was an observational, multicentre, prospective cohort study investigating the baseline characteristics, clinical course of disease and use of antifibrotic treatment in Italian patients with idiopathic pulmonary fibrosis (IPF).
Methods: Patients aged ≥ 40 years diagnosed with IPF within the previous 3 months at 20 Italian centres were con- secutively enrolled and followed up for 12 months, with evaluations at 3, 6, 9 and 12 months. The primary objective was to describe the clinical course of IPF over 12 months of follow-up, including changes in lung function measured by % predicted forced vital capacity (FVC% predicted).
Results: 209 patients (82.3% male, mean age 69.54 ± 7.43 years) were enrolled. Mean FVC% predicted was rela- tively preserved at baseline (80.01%). The mean time between IPF diagnosis and initiation of antifibrotic therapy was 6.38 weeks; 72.3% of patients received antifibrotic therapy within the first 3 months of follow-up, and 83.9% within 12 months of follow-up. Mean FVC% predicted was 80.0% at baseline and 82.2% at 12 months, and 47.4% of patients remained stable (i.e. had no disease progression) in terms of FVC% predicted during the study.
Conclusions: FIBRONET is the first prospective, real-life, observational study of patients with IPF in Italy. The short time between diagnosis and initiation of antifibrotic therapy, and the stable lung function between baseline and 12 months, suggest that early diagnosis and prompt initiation of antifibrotic therapy may preserve lung function in patients with IPF.
Trial registration: NCT02803580

Relevance
Long-term data on the natural course of IPF in Italy are scarce, and there is limited information on patient char- acteristics and disease management. However, a grow- ing body of real-world evidence from Italy suggests that pirfenidone and nintedanib can attenuate the decline in lung function in patients with IPF, and data from other real-world studies in Europe and Australia suggest that antifibrotic treatment may prolong survival. Integrating evidence from randomised controlled trials with real-life evidence has become increasingly important for the respiratory physician, since real-life studies are not limited by strict selection criteria and therefore approximate the general patient population more accurately. Adding to the existing body of real- life evidence in Italy, the primary objective of this study was to describe the baseline characteristics, the clinical course of the disease (in terms of changes in lung func- tion, including forced vital capacity [FVC]% predicted) and the use of antifibrotic treatment in a group of Italian patients with IPF, during 12 months of observation.