Clinical course of IPF in Italian patients during 12\xa0months of observation: results from the FIBRONET observational study

V Poletti,C Vancheri,A Pesci,C Albera,R R Metella,G Crespi,S Rizzoli,S Harari,B Campolo

doi:10.1186/s12931-021-01643-w

Abstract

BackgroundFIBRONET 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).MethodsPatients aged ≥ 40 years diagnosed with IPF within the previous 3 months at 20 Italian centres were consecutively 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).Results209 patients (82.3% male, mean age 69.54 ± 7.43 years) were enrolled. Mean FVC% predicted was relatively 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.ConclusionsFIBRONET 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

Highlights

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)
The most common Idiopathic interstitial pneumonia (IIP) is idiopathic pulmonary fibrosis (IPF) [1], a progressive, and fatal, disease characterised by progressive fibrosis of the lung parenchyma and subsequent decline in lung function, and defined by a usual interstitial pattern on high-resolution computed tomography (HRCT) [3,4,5]
Inclusion and exclusion criteria Patients were included if they were aged ≥ 40 years, with a diagnosis of IPF confirmed by a physician during the previous 3 months [3] and an assessment of IPF based on HRCT, or HRCT and surgical lung biopsy if required and available

Summary

Introduction

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). The most common IIP is idiopathic pulmonary fibrosis (IPF) [1], a progressive, and fatal, disease characterised by progressive fibrosis of the lung parenchyma and subsequent decline in lung function, and defined by a usual interstitial pattern on high-resolution computed tomography (HRCT) [3,4,5]. Diagnosing IPF in clinical practice can be challenging, as symptoms often appear similar to those of more common respiratory diseases, such as chronic obstructive pulmonary disease. This contributes to a delay in diagnosis, which is usually made 6–24 months after initial symptoms [11,12,13], but can be made even later (> 3 years) [14]. According to one survey of 290 respiratory physicians in Europe, 40% of patients with a confirmed diagnosis of IPF in Italy, Spain, France, Germany and the UK do not receive treatment with an approved antifibrotic [18]

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