Does timeliness of diagnosis influence survival and treatment response in idiopathic pulmonary fibrosis? Real- world results from the EMPIRE registry

Aims: Many patients with idiopathic pulmonary fibrosis (IPF) have a delay in diagnosis and thus also in treatment. We investigated whether such delay influenced survival and treatment response to pirfenidone and nitedanib. Methods: The retrospective study used data from European Multipartner IPF registry (EMPIRE). 1170 IPF patients were divided to the 2 groups: 1) patients diagnosed within (824) and 2) patients diagnosed after 12 months (346) from initiation of symptoms. The groups did not differ in demographic data, and in proportion of the patients treated with antifibrotic drugs. Survival from diagnosis up to 120 months was evaluated by Kaplan-Meier curve. The vital capacity (VC) initial values (% PV) and VC decline (l) in 6, 12, and 18 months of follow-up were evaluated and compared between both groups using t-test. Results: VC at the time of diagnosis was higher in earlier diagnosed group (2.72 l (2.63; 2.80) versus 2.49 l (2.37; 2.61); p=0.002). The Kaplan-Meier survival curve showed longer median survival in the earlier diagnosed patients (74.4 vs. 50.9 months (P=0.001)). The patients treated with pirfenidone showed longer median survival versus those without antifibrotic treatment (46.9 vs. 39.6 months; p=0.014), nintedanib showed similar tendency. Treatment with antifibrotic drugs similarly reduced decline of VC and TLCO in both early and later diagnosed patients. Conclusion: The patients with IPF diagnosed within 1 year from symptoms have longer survival than those diagnosed later. Antifibrotic treatment in real-world prolongs survival in IPF and slows down decline of lung function irrespective of timeliness of diagnosis.