V Poletti1,2, C Vancheri3, C Albera4, S Harari5,6, A Pesci7, R R Metella8, B Campolo9, G Crespi9, S Rizzoli10. 1. Department of Diseases of the Thorax, Ospedale GB Morgagni, Forlì, Italy. 2. Department of Respiratory Diseases & Allergy, Aarhus University Hospital, Aarhus, Denmark. 3. Regional Referral Centre for Rare Lung Diseases-University Hospital "Policlinico G. Rodolico", Department of Clinical and Experimental Medicine, University of Catania, Catania, Italy. vancheri@unict.it. 4. S.C. Pneumologia U., A.O.U. Città Della Scienza E Della Salute (Molinette), University of Torino, Torino, Italy. 5. Department of Clinical Sciences and Community Health, University of Milan, Milan, Italy. 6. Department of Medicine, Ospedale San Giuseppe MultiMedica IRCCS, Milan, Italy. 7. Ospedale San Gerardo, ASST Monza, Monza, Italy. 8. Dipartimento di Scienze Mediche Chirurgiche e Neuroscienze, Università degli Studi di Siena, Siena, Italy. 9. Boehringer Ingelheim, Milan, Italy. 10. MediNeos Observational Research, Modena, Italy.
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.
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.
Authors: Joshua Mooney; Eunice Chang; Deepa Lalla; Elya Papoyan; Karina Raimundo; Sheila R Reddy; John Stauffer; Tingjian Yan; Michael S Broder Journal: Ann Am Thorac Soc Date: 2019-03
Authors: Katarzyna Górska; Marta Maskey-Warzęchowska; Małgorzata Barnaś; Adam Białas; Adam Barczyk; Hanna Jagielska-Len; Ewa Jassem; Aleksander Kania; Katarzyna Lewandowska; Sebastian Majewski; Magdalena M Martusewicz-Boros; Wojciech J Piotrowski; Alicja Siemińska; Krzysztof Sładek; Małgorzata Sobiecka; Marzena Trzaska-Sobczak; Witold Tomkowski; Beata Żołnowska; Rafał Krenke Journal: Ther Adv Chronic Dis Date: 2022-08-22 Impact factor: 4.970