Abstract
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.
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