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Background: A correlation of adverse events (AE), and the efficacy of EGFR TKIs in lung cancer patients (pts) is known. Similar AE are seen in IPF pts treated with triple TKI nintedanib (N) and pirfenidone (P). Post hoc analysis to assess the correlation between clinically outcomes in IPF pts from the real-world EMPIRE registry and AE to antifibrotics. Methods The analysis was performed 6 months after the initiation of antifibrotics according to AE. Correlation with mortality and progression-free survival-PFS, were assessed by the Kaplan–Meier survival function, correlation with AE by the Cox proportional hazards regression analysis. Results: Out of 1,233 pts, 698 were treated with P and 535 with N; AE were observed in 96 pts (7.8%): dyspepsia in 2.1%, elevated liver enzymes in 1.6%, diarrhoea in 0.7%, nausea in 1.1%, photosensitivity,rash,vomiting in 0.3% each, fatigue in 0.2%. The occurrence of AE correlated with the PFS, for all pts (p= 0.004) and for treated with N (p= 0.015), but not mortality. PFS was significantly better in the group of pts with no AE. Fatigue in all pts (adjusted HR 6.0; 1.5–24.8;p= 0.012), in treated with P(5.0; 1.2–20.5;p= 0.027), nausea in pts with N (6.8;1.6–28.8;p= 0.010) correlated with higher mortality. The effect of AE on PFS were noted in all pts for nausea (2.2; 1.2–3.8; p= 0.006) and rash (4.1;1.5–11.0;p= 0.005); in pts on P for rash (4.2;1.5–11.2;p= 0.005);in pts on N for nausea (3.5;1.6–7.5; p= 0.001). Conclusion: The occurrence of AE correlates with PFS, but not with mortality. The occurrence of individual AE is associated with a poorer prognosis of pts.

European-Respiratory-Society (ERS) International Congress -- SEP 28-OCT 02, 2019 -- Madrid, SPAIN, WOS: 000507372406214, [No abstract available], European Respiratory Soc, Boehringer IngelheimBoehringer Ingelheim, The EMPIRE registry is supported by Boehringer Ingelheim

EMPIRE is an international, multicentre registry of idiopathic pulmonary fibrosis (IPF) patients in Central and Eastern Europe. Two approved treatments are available, nintedanib and pirfenidone. Nintedanib may increase the risk of bleeding and patients treated with anticoagulants were excluded from clinical trials. Our aim was to examine bleeding risk with current treatments in EMPIRE patients. IPF patients (N=1528) were divided into four groups: Group A (no anticoagulant or antiplatelet therapy; N=984); Group B (anticoagulant treatment; N=131); Group C (antiplatelet treatment; N=371); Group D (anticoagulant and antiplatelet treatment; N=42). Nintedanib was used in 203 (13.7%), pirfenidone in 283 (19.1%) patients. FVC was similar in all groups; only 5-10% of patients had FVC In EMPIRE, patients on anticoagulant medications are less frequently receiving nintedanib therapy, which might be due to the mode of action of nintedanib. Overall, the incidence of bleeding events was very low.

Idiopathic pulmonary fibrosis (IPF) is a rare, deadly lung disease. Diagnosis and treatment possibilities are dependent on countries’ health systems. Comparison between countries is difficult due to data heterogenity. Our aim was to analyze IPF patients in Central and Eastern Europe using the uniform data from European Multipartner IPF registry (EMPIRE) involving 7 countries. Newly diagnosed IPF patients (n=1114) from Czech Republic (n=554, 70.0% men (M)), Turkey (n=197, 74.2% M), Poland (n=140, 72.9% M), Slovakia (n=89, 70.8% M), Hungary (n=88, 48.9% M), Serbia (n=28, 42.9% M) and Croatia (n=21, 90.5% M) were included. Baseline characteristics, lung function, HRCT pattern, comorbidities and treatment data were analyzed. Patients were significantly older in Poland than in Turkey and Serbia. Former smokers were most common in Croatia (95.5%), least frequent in Serbia (35.7%). Baseline FVC was between 50-80% in 50.9% and >80% in 42.2% of patients. Most IPF patients with FVC>80% were registered in Poland (61%) and Slovakia (58%); the lowest in Croatia (32%) and Turkey (33%). Typical UIP pattern was present in 75.4% of all patients, ranging from 96.4% (Turkey) to 57.1% (Serbia). Most common comorbidities were cardiovascular and gastrointestinal diseases, with huge intercountry differences. Majority of patients did not receive antifibrotic therapy; only 31.5% ever used pirfenidone (range 4.3-46.7% between countries) and 22.1% nintedanib (range 0-71.4% between countries) treatment. Significant differences in IPF patient characteristics and access to antifibrotic therapies exist in EMPIRE countries which needs further investigation and strategies to improve patient care in this region.

Aims: Idiopathic pulmonary fibrosis (IPF) clinical behaviour is mostly known only from clinical trials and from single center or country experiences. We aim to show real world of IPF in Central and Eastern Europe on the data from European Multipartner IPF registry (EMPIRE) which currently involves 8 European countries. Patients and methods: The analysis was performed on 2048 patients (626 F; 1422 M; 834 (40.7%) non-smokers; 817 from the Czech republic, 377 Turkey, 366 Poland, 182 Hungary, 159 Slovakia, 84 Serbia, 45 Croatia, 18 Israel). Baseline demographic data, lung function, and HRCT patterns were collected and their relations to survival were statistically evaluated. Results: Median age was 67.7 years (range 50.1–81.9), symptom duration 11.0 (range 1.0-50.0) months. Median forced vital capacity (FVC) was 76.2% (range 47.1-111.6) and transfer factor (TLCO) 47% (range 22.2-78.6). Typical HRCT pattern (definite UIP) was seen in 1550 patients (75.5%), and atypical (possible or inconsistent with UIP) in 491 (24.5%). 662 patients were on pirfenidone and 481 on nintedanib. Survival was significantly longer in females (p ˂0.001), in the patients with higher FVC and TLCO (p ˂0.001), non-smokers (p=0.021) and the patients with atypical HRCT presentation (p=0.011). Also pirfenidone (p=0.033) and nintedanib (p ˂0.001) treatment offered survival advantage compared to any other or no therapy. Conclusion: Better outcome was observed in females and in the patients with better initial FVC and TLCO, with atypical HRCT pattern and in those on anti-fibrotic treatment. The EMPIRE registry can show IPF in real world and prove effectivity of anti-fibrotic treatment across the diversity of IPF compared to randomized clinical trials.

Key feature of IPF is decline in forced vital capacity (FVC) and CO diffusion (DLco), but data on clinical determinants of this change in patients who have established disease are scarce. For a given patient it is unknown if different natural histories represent distinct phenotypes of IPF or if geographic, ethnic or other factors might have influence on progression. The EMPIRE (European Multipartner IPF Registry) is a multicountry IPF register in the CEE region. Out of 1229 patients from the registry 324 with available data on FVC and DLco at inclusion and at 6 months rapid decliners (RD: FVC >10% or DLco >10% in 6 months) and controls (C) were included into the analysis. Clinical characteristics at inclusion were analyzed between groups to find clinical determinants of RD. Average age was 67,4±9.7 years, more males were affected than females (male:female 221:103) most patients had classic signs of UIP on HRCT. Most common comorbidity was hypertension (50.9%), coronary heart disease (20.5%), diabetes (20.1%) and hyperlipidaemia (20.1%). RD of FVC was present in 33.7%, while RD of DLco in 53.3% of patients. Comparison of RD and C patients for smoking status, dyspnoe severity, comorbidities, HRCT pattern, lung function parameters at inclusion, 6 minute walk distance or the presence of pulmonary hypertension did not show any difference between groups. The rate of change in FVC or DLco among IPF patients is highly variable, with 34-53 % of patients showing increased rates of decline in FVC and/or DLco. No clinical characteristics could be identified which can predict rapid decline using the data of 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.

AIMS: Idiopathic pulmonary fibrosis (IPF) is a severe lung disease with mean survival of 2.5 years. Delay in diagnosis and treatment probably decreases a survival in IPF. Our study aims to investigate influence of time from the first symptoms to diagnosis on the survival. CONCLUSIONS: Our results show that earlier diagnosis and treatment may increase survival in IPF. Early diagnosis and treatment does matter in prognosis of IPF patients and thus maximal effort should be generated to get the patients diagnosed and treated as early as possible.