Abstract
Background: Therapies that slow IPF progression are now available and recent studies suggest that the use of anti-fibrotic therapy may reduce IPF mortality. Our aim was to evaluate whether, to what extent and for which factors the survival of IPF in a real life setting has changed in the last 15 years. Methods: Historical eye is an observational study of a large cohort of consecutive IPF patients diagnosed and treated in a referral centre for ILDs with prospective intention. Findings: The study comprised 634 patients. The year 2012 identifies the time point of mortality shift ( HR 0.58, CI 0.46-0.63, p <0.001). In the more recent cohort more patients had a better preserved lung function, underwent cryobiopsy instead of surgery and were treated with antifibrotics. Highly significant negative prognostic factors were lung cancer (HR 4.46, 965% CI 3.3-6, p<0.001), hospitalizations (HR 8.37, 95% CI 6.5-10.7, p<0.001) and acute exacerbations HR 8.37 (95% CI 6.52-10.7), p<0.001. Average antifibrotic treatment effect estimated using propensity score matching showed a significant effect in the reduction of all causes mortality (ATE coeff -0.23, SE 0.04, p<0.001), acute exacerbations (ATE coeff -0.15, SE 0.04, p<0.001) and hospitalizations (ATE coeff -0.15, SE 0.04, p<0.001), but no effect on lung cancer risk (ATE coeff -0.03, SE 0.03, p=0.4). Interpretation: Antifibrotic drugs significantly impact hospitalizations, acute exacerbations and IPF survival. After the introduction of cryobiopsy and antifibrotic drugs the prognosis of IPF patients has significantly improved together with our ability to detect IPF at an earlier stage. Funding Statement: This work was sponsored by F. Hoffmann-La Roche Ltd./Genentech, Inc. and Associazione Morgagni Malattie Polmonari, Forli, IT Declaration of Interests: Sara Tomassetti received a grant from Roche and speaker’s fees from Roche and Boehringher Ingelheim. All other authors have no competing interests to declare. Ethics Approval Statement: The study was approved by the Comitato Etico di area vasta ROMagna, Italy (CEROM approval protocol number 8571/2017). Patients provided informed consent according to current local and national legislation.
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