Abstract 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 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). Results: 209 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. 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. Keywords: Antifibrotic therapy; Idiopathic pulmonary fibrosis; Italy; Lung function; Nintedanib; Observational; Pirfenidone; Real-world. Conflict of interest statement BC was an employee of Boehringer Ingelheim (Italy) at the time of this study. GC is an employee of Boehringer Ingelheim. SR is an employee of MediNeos Observational Research (Modena, Italy). AP has received personal (speaker/advisory board) fees from Boehringer Ingelheim and Roche, and his research activity is partially supported by project Premia. SH has acted as a clinical trial investigator, participated in scientific advisory boards, and delivered lectures for Boehringer Ingelheim, Roche and Actelion. He has also received research grants from Boehringer Ingelheim. CA has acted as a clinical trial investigator for Boehringer Ingelheim and Roche, participated in scientific advisory boards for Boehringer Ingelheim, Roche, MSD, Fibrogen and GSK, and received research grants from Boehringer Ingelheim and Roche. CV has received research grants and personal fees from Boehringer Ingelheim and Roche. VP has received personal fees from Boehringer Ingelheim. RRM has nothing to disclose. Figures Fig. 1 Fig. 1 FVC% predicted at baseline. Percentages… Fig. 2 Fig. 2 Mean change in FVC% predicted… Cited by 1 article Patient Reported Experiences and Delays During the Diagnostic Pathway for Pulmonary Fibrosis: A Multinational European Survey. van der Sar IG, Jones S, Clarke DL, Bonella F, Fourrier JM, Lewandowska K, Bermudo G, Simidchiev A, Strambu IR, Wijsenbeek MS, Parfrey H. Front Med (Lausanne). 2021 Aug 4;8:711194. doi: 10.3389/fmed.2021.711194. eCollection 2021. PMID: 34422866 Free PMC article. References Demedts M, Costabel U. ATS/ERS international multidisciplinary consensus classification of the idiopathic interstitial pneumonias. Eur Respir J. 2002;19:794–796. doi: 10.1183/09031936.02.00492002. - DOI - PubMed Travis WD, Costabel U, Hansell DM, King TE, Jr, Lynch DA, Nicholson AG, Ryerson CJ, Ryu JH, Selman M, Wells AU, et al. An official American Thoracic Society/European Respiratory Society statement: Update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med. 2013;188:733–748. doi: 10.1164/rccm.201308-1483ST. - DOI - PMC - PubMed Raghu G, Remy-Jardin M, Myers JL, Richeldi L, Ryerson CJ, Lederer DJ, Behr J, Cottin V, Danoff SK, Morell F, et al. Diagnosis of idiopathic pulmonary fibrosis. An official ATS/ERS/JRS/ALAT clinical practice guideline. Am J Respir Crit Care Med. 2018;198:e44–e68. doi: 10.1164/rccm.201807-1255ST. - DOI - PubMed Karimi-Shah BA, Chowdhury BA. Forced vital capacity in idiopathic pulmonary fibrosis–FDA review of pirfenidone and nintedanib. N Engl J Med. 2015;372:1189–1191. doi: 10.1056/NEJMp1500526. - DOI - PubMed Paterniti MO, Bi Y, Rekic D, Wang Y, Karimi-Shah BA, Chowdhury BA. Acute exacerbation and decline in forced vital capacity are associated with increased mortality in idiopathic pulmonary fibrosis. Ann Am Thorac Soc. 2017;14:1395–1402. doi: 10.1513/AnnalsATS.201606-458OC. - DOI - PubMed
Clinical course of IPF in Italian patients during 12 months of observation: results from the FIBRONET observational study.
Albera C;
2021-01-01
Abstract
Abstract 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 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). Results: 209 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. 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. Keywords: Antifibrotic therapy; Idiopathic pulmonary fibrosis; Italy; Lung function; Nintedanib; Observational; Pirfenidone; Real-world. Conflict of interest statement BC was an employee of Boehringer Ingelheim (Italy) at the time of this study. GC is an employee of Boehringer Ingelheim. SR is an employee of MediNeos Observational Research (Modena, Italy). AP has received personal (speaker/advisory board) fees from Boehringer Ingelheim and Roche, and his research activity is partially supported by project Premia. SH has acted as a clinical trial investigator, participated in scientific advisory boards, and delivered lectures for Boehringer Ingelheim, Roche and Actelion. He has also received research grants from Boehringer Ingelheim. CA has acted as a clinical trial investigator for Boehringer Ingelheim and Roche, participated in scientific advisory boards for Boehringer Ingelheim, Roche, MSD, Fibrogen and GSK, and received research grants from Boehringer Ingelheim and Roche. CV has received research grants and personal fees from Boehringer Ingelheim and Roche. VP has received personal fees from Boehringer Ingelheim. RRM has nothing to disclose. Figures Fig. 1 Fig. 1 FVC% predicted at baseline. Percentages… Fig. 2 Fig. 2 Mean change in FVC% predicted… Cited by 1 article Patient Reported Experiences and Delays During the Diagnostic Pathway for Pulmonary Fibrosis: A Multinational European Survey. van der Sar IG, Jones S, Clarke DL, Bonella F, Fourrier JM, Lewandowska K, Bermudo G, Simidchiev A, Strambu IR, Wijsenbeek MS, Parfrey H. Front Med (Lausanne). 2021 Aug 4;8:711194. doi: 10.3389/fmed.2021.711194. eCollection 2021. PMID: 34422866 Free PMC article. References Demedts M, Costabel U. ATS/ERS international multidisciplinary consensus classification of the idiopathic interstitial pneumonias. Eur Respir J. 2002;19:794–796. doi: 10.1183/09031936.02.00492002. - DOI - PubMed Travis WD, Costabel U, Hansell DM, King TE, Jr, Lynch DA, Nicholson AG, Ryerson CJ, Ryu JH, Selman M, Wells AU, et al. An official American Thoracic Society/European Respiratory Society statement: Update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med. 2013;188:733–748. doi: 10.1164/rccm.201308-1483ST. - DOI - PMC - PubMed Raghu G, Remy-Jardin M, Myers JL, Richeldi L, Ryerson CJ, Lederer DJ, Behr J, Cottin V, Danoff SK, Morell F, et al. Diagnosis of idiopathic pulmonary fibrosis. An official ATS/ERS/JRS/ALAT clinical practice guideline. Am J Respir Crit Care Med. 2018;198:e44–e68. doi: 10.1164/rccm.201807-1255ST. - DOI - PubMed Karimi-Shah BA, Chowdhury BA. Forced vital capacity in idiopathic pulmonary fibrosis–FDA review of pirfenidone and nintedanib. N Engl J Med. 2015;372:1189–1191. doi: 10.1056/NEJMp1500526. - DOI - PubMed Paterniti MO, Bi Y, Rekic D, Wang Y, Karimi-Shah BA, Chowdhury BA. Acute exacerbation and decline in forced vital capacity are associated with increased mortality in idiopathic pulmonary fibrosis. Ann Am Thorac Soc. 2017;14:1395–1402. doi: 10.1513/AnnalsATS.201606-458OC. - DOI - PubMed
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