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Introduction: The prevalence and natural history of PF-ILDs and their response to commonly used therapies in real-life cohorts is largely unknown. Aims: We aim to describe the prevalence, management and outcomes of PF-ILD pts attending two Italian referral centers (San Gerardo Hospital, Monza and San Giuseppe Hospital, Milan) from Jan 1st 2011 to Jul 31st 2019. Methods: From a cohort of non-IPF fibrosing ILD pts with at least 2-yr follow-up, we selected only those with progressive disease, defined as per INBUILD trial (Flaherty,K.R. et al.NEJM2019;381:1718–27) collecting their demographic, clinical and functional data. Results: Out of 245 non-IPF fibrosing ILD pts, 75(31%) were classified as PF-ILD (median age 66 yrs, 60% males), most frequently iNSIP(28%) followed by CTD-ILD(20%), chronic HP and sarcoidosis (17% each). Disease progression occurred after a median of 18 months from ILD diagnosis. The vast majority(93%) of PF-ILD pts received prednisolone, alone(40%) or associated with other immunosuppressants(52%); 35% of treated pts developed treatment-related adverse events. After ILD progression, median survival was 3yrs, with a 2-and 3-yr mortality rate of 4% and 20% respectively (Fig1). Conclusions: In a real-life setting approximately one third of fibrosing ILDs pts showed a progressive course despite treatment. Studies aimed to better phenotype this subgroup of pts are most warranted.

Introduction: Idiopathic Pulmonary Fibrosis (IPF) is a rare disease associated with poor survival and increasing disability. Aims and Objectives: FIBRONET study was designed to provide information on IPF characteristics and progression and use of antifibrotic treatment in newly diagnosed patients. Methods: FIBRONET is an observational, multicenter, prospective cohort study on ≥40 years-old Italian newly-diagnosed IPF patients observed for 12 months. Results: 209 consecutive patients were enrolled [mean±SD age: 69.54±7.43 years, 173 (82.8%) males]. At baseline 140 (71.4%) and 59 (30.1%) out of 196 evaluable patients had forced vital capacity (FVC) ≥ 70% and ≥ 90% predicted, respectively; 184 (88.0%) had ≥1 IPF symptoms; mean of walked distance during 6 minutes walking test (6MWT) was 395.7 meters. On average (±SD), 6.38 (±7.06) weeks elapsed between IPF diagnosis and the start of antifibrotic treatment; after 1 year from diagnosis, 83.9% of patients was on treatment. According to FVC % predicted relative change over 12 months, 70 (52.6%) and 63 (47.4%) patients were classified as decliners or not decliners, respectively. At 12-month follow up IPF symptoms frequency was 45.4% and mean distance during 6MWT was 411.7 meters. Fifteen patients had 18 exacerbations during the study; the 1-year probability of exacerbation was 6.5%. Conclusions: For the first time, FIBRONET described the clinical course of IPF in a large cohort of newly-diagnosed Italian patients in a real-life setting. Forty-seven percent of patients remained stable or improved FVC % predicted after 12 months. This could be reasonably related to the early start of antifibrotic treatment after diagnosis.