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Anticoagulant Use and Bleeding Risk in Central European Patients with Idiopathic Pulmonary Fibrosis (IPF) Treated with Antifibrotic Therapy: Real-World Data from EMPIRE
(Adis Int Ltd, 2020) Kolonics-Farkas, Abigel M.; Sterclova, Martina; Mogulkoc, Nesrin; Kus, Jan; Hajkova, Marta; Muller, Veronika; Vasakova, Martina
Introduction Nintedanib, a tyrosine kinase receptor inhibitor, may be associated with increased bleeding risk. Thus, patients with an inherited predisposition to bleeding, or those receiving therapeutic doses of anticoagulants or high-dose antiplatelet therapy, have been excluded from clinical trials of nintedanib in idiopathic pulmonary fibrosis (IPF). Objective Our objective was to examine real-world bleeding events in patients with IPF treated with antifibrotics, including those receiving anticoagulants and/or antiplatelet therapy. Methods the European MultiPartner IPF Registry (EMPIRE) enrolled 2794 patients with IPF: group A (1828: no anticoagulant or antiplatelet treatment), group B (227: anticoagulant treatment), group C (659: antiplatelet treatment), and group D (80: anticoagulant and antiplatelet treatment). Overall, 673 (24.1%) received nintedanib and 933 (33.4%) received pirfenidone. Bleeding events and their relationship to antifibrotic and anticoagulation treatment were characterized. Results Group A patients, versus those in groups B, C, and D, were typically younger and generally had the lowest comorbidity rates. A higher proportion of patients in groups A and C, versus group B, received nintedanib. Pirfenidone, most common in group D, was more evenly balanced across groups. in patients with reported bleeding events, seven of eight received nintedanib (groups A, C, and D). Bleeding incidence was 3.0, 0, 1.3, and 18.1 per 10,000 patient-years (groups A, B, C, and D, respectively). Conclusion Real-world data from EMPIRE showed that patients on anticoagulant medications received nintedanib less frequently, perhaps based on its mechanism of action. Overall, bleeding incidence was low (0.29%: nintedanib 0.25%; pirfenidone 0.04%) and irrespective of anticoagulant or antiplatelet therapy received (P = 0.072).
Assessment of right ventricular function in patients with pulmonary arterial hypertension-congenital heart disease and repaired and unrepaired defects: Correlation among speckle tracking, conventional echocardiography, and clinical parameters
(Turkish Soc Cardiology, 2020) Kemal, Hatice S.; Kayikcioglu, Meral; Nalbantgil, Sanem; Can, Levent Hurkan; Mogulkoc, Nesrin; Kultursay, Hakan
Objective: the purpose of this study is to compare the analysis of right ventricular (RV) free wall strain via 2D speckle tracking echocardiography with conventional echocardiography and clinical parameters in patients with pulmonary arterial hypertension associated with congenital heart disease (PAH-CHD) receiving specific treatment. This study also aims to describe the differences between patients with repaired and unrepaired defects. Methods: This prospective study included 44 adult patients with PAH-CHD who were receiving PAH-specific treatment in a single center. This study excluded patients with complex congenital heart disease. the authors studied the conventional echocardiographic parameters, such as RV fractional area change (FAC), tricuspid annular plane systolic excursion (TAPSE), right atrial (RA) area, Tricuspid S', and hemodynamic parameters, such as functional class, 6-minute walking distance (6MWD), and N-terminal pro-brain natriuretic peptide (NT-proBNP) levels. Results: the mean age of participants was 33.8 +/- 11.6 years, and 65.9% of participants were female. the mean RV free wall strain was -14.8 +/- 4.7%. Majority of the patients belonged to WHO functional class 2 (61.4%) with a mean NT-proBNP level of 619.2 +/- 778.4 and mean 6MWD of 400.2 +/- 86.9 meters. During the follow-up of 30.8 +/- 9.0 months, 6 patients (13.6%) developed clinical right heart failure, whereas 9 (20.5%) of them died. There was a positive and significant correlation between RV free wall strain and WHO functional class (r=0.320, p=0.03), whereas there was a negative correlation between RV free wall strain and FAC (r=-0.392, p=0.01), TAPSE (r=-0.577, p=0.0001), and Tricuspid S' (r=-0.489, p=0.001). There was no significant correlation of RV free wall strain with either RA area or 6MWD. Patients with repaired congenital heart defects had worse RV functional parameters and RV free wall strain than patients with unrepaired defects. Conclusion: the assessment of RV free wall strain via 2D speckle tracking echocardiography is a feasible method and correlates well with conventional echocardiography and clinical parameters in patients with PAH-CHD receiving specific treatment.
The association between white blood cell count and outcomes in patients with idiopathic pulmonary fibrosis
(W B Saunders Co Ltd, 2020) Nathan, Steven D.; Brown, A. Whitney; Mogulkoc, Nesrin; Soares, Flavia; Collins, Ashley C.; Cheng, Joyce; Barnett, Scott D.
Background: the course of idiopathic pulmonary fibrosis (IPF) is uncertain with variable patterns of disease progression. We sought to evaluate the prognostic utility of the WBC, a routinely performed lab test, in a well-defined cohort of outpatient IPF subjects. Methods: We reviewed IPF patient records from two independent ILD centers (Inova Fairfax in Falls Church, VA, USA and Ege University Hospital in Izmir, Turkey) between 2007 and 2018. Demographics, CBC data, and patient outcomes were obtained. Survival differences were analyzed. Results: There were 436 IPF outpatients in the cohort with a median WBC of 8.9 x 10(9) cells per liter. For pragmatic purposes, patients were categorized into two groups, WBC >= 9 or WBC <9. Patients with WBC <9 had a median transplant-free survival of 50.5 months from the time of the CBC, compared to 32.4 months for those with WBC >= 9 (p < 0.0001). the association between WBC and attenuated survival remained significant after adjusting for GAP stage, steroid use, and antifibmtic use when WBC was analyzed both as a continuous (HR: 1.11; 95% CI: 1.05-1.17) and a dichotomized variable (high (WBC >= 9) vs. low (WBC <9), (HR: 1.53; 95% CI:1.09-2.15). WBC and absolute neutrophil count (ANC) were highly correlated suggesting that PMNs account for most of this association (r = 0.92). Conclusions: Baseline WBC may impart important and readily available prognostic information in outpatients with IPF. Further studies are warranted to validate this as a potential biomarker for IPF, as well as to define the biologic basis for the association.
Bleeding risk in IPF patients treated with different anticoagulants: Real world data from the European MultiPartner IPF Registry (EMPIRE)
(European Respiratory Soc Journals Ltd, 2018) Kolonics-Farkas, Abigel Margit; Sterclova, Martina; Mogulkoc, Nesrin; Kus, Jan; Mueller, Veronika; Hajkova, Marta; Jovanovic, Dragana; Tekavec-Trkanjec, Jasna; Kramer, Mordechai; Svoboda, Michal; Janotova, Marketa; Vasakova, Martina
A case of pulmonary capillary haemangiomatosis (PCH) diagnosed by lung biopsy
(European Respiratory Soc Journals Ltd, 2013) Mogulkoc, Nesrin; Coskun, Aysin Sakar; Bishop, Paul
Comorbid conditions in IPF patients; their frequencies and impacts on survival
(European Respiratory Soc Journals Ltd, 2018) Koc, Aysu Sinem; Mogulkoc, Nesrin; Bishop, Paul
Comorbidity burden and survival in patients with idiopathic pulmonary fibrosis: the EMPIRE registry study
(Bmc, 2022) Jovanovic, Dragana M.; Sterclova, Martina; Mogulkoc, Nesrin; Lewandowska, Katarzyna; Muller, Veronika; Hajkova, Marta; Studnicka, Michael
Background Patients with idiopathic pulmonary fibrosis (IPF) frequently have multiple comorbidities, which may influence survival but go under-recognised in clinical practice. We therefore report comorbidity, antifibrotic treatment use and survival of patients with IPF observed in the multi-national EMPIRE registry. Methods For this prospective IPF cohort, demographics, comorbidities, survival and causes of death were analysed. Comorbidities were noted by the treating physician based on the patient's past medical history or as reported during follow-up. Comorbidities were defined as prevalent when noted at enrolment, or as incident when recorded during follow-up. Survival was analysed by Kaplan-Meier estimates, log-rank test, and Cox proportional hazards models. Hazard ratios (HR) were adjusted for gender, age, smoking status and FVC at enrolment. Results A population of 3,580 patients with IPF from 11 Central and Eastern European countries was followed every 6 months for up to 6 years. At enrolment, 91.3% of patients reported at least one comorbidity, whereas more than one-third (37.8%) reported four or more comorbidities. Five-year survival was 53.7% in patients with no prevalent comorbidities, whereas it was 48.4%, 47.0%, 43.8% and 41.1% in patients with 1, 2, 3 and >= 4 comorbidities, respectively. The presence of multiple comorbidities at enrolment was associated with significantly worse survival (log-rank test P = 0.007). Adjusted HRs indicate that risk of death was increased by 44% in patients with IPF reporting >= 4 comorbidities at baseline compared with no comorbidity (P = 0.021). The relationship between number of comorbidities and decreased survival was also seen in patients receiving antifibrotic treatment (63% of all patients; log-rank test P < 0.001). Comorbidity as cause of death was identified in at least 26.1% of deaths. Conclusions The majority of patients with IPF demonstrate comorbidities, and many have comorbidity-related deaths. Increasing numbers of comorbidities are associated with worse survival; and this pattern is also present in patients receiving antifibrotic therapy.
CONJUNCTIVAL BIOPSY AS A FIRST CHOICE TO CONFIRM A DIAGNOSIS OF SARCOIDOSIS
(Mattioli 1885, 2016) Ekren, Pervin Korkmaz; Mogulkoc, Nesrin; Toreyin, Zehra Nur; Egrilmez, Sait; Veral, Ali; Akalin, Taner; Bacakoglu, Feza
Background: Sarcoidosis is a granulomatous systemic disease of unknown aetiology. The diagnosis needs histological confirmation of the presence of non-caseating granulomata. One option is a conjunctival biopsy. The aims of this study were to evaluate conjunctival biopsy for the diagnosis of sarcoidosis with respect to its sensitivity and to assess its cost effectiveness by comparison with other histopathological diagnostic procedures. Methods: Patients were identified from the database of the Interstitial Lung Disease Clinic (ILDC) of the Chest Department of Ege University Hospital from May 2008 to June 2014. The patients who had biopsy procedures performed for the definitive diagnosis of sarcoidosis were assessed. Their diagnostic procedures and the cost of procedures were recorded. The cost per positive result for each procedure was calculated. Results: In total, 280 patients were followed up with a diagnosis of sarcoidosis, of whom 174 had histological confirmation; these constitute the study population. There were 127 females and 47 males with a median age of 46 years (range 14-78 years). Forty three patients had conjunctival biopsy and we could establish a diagnosis in 54% of these by means of conjunctival biopsy. Moreover, we showed that this biopsy can provide positive result for sarcoidosis patients who lack abnormal eye findings. Additionally, it is cost effective approach and without complications. Conclusion: This study re-asserts the value of conjunctival biopsy, which was described in the past but is not commonly used nowadays. In the presence of suggestive clinic and radiologic findings, we recommend conjunctival biopsy as the first choice for the histopathological confirmation of sarcoidosis.
Correlation of response to antifibrotic treatment with adverse events to antifibrotic drugs in IPF patients from the real-world EMPIRE registry
(European Respiratory Soc Journals Ltd, 2020) Jovanovic, Dragana; Sterclova, Martina; Mogulkoc, Nesrin; Lewandowska, Katarzyna; Muller, Veronika; Hajkova, Marta; Kaeva, Biserka Jovkovska
[No Abstract Available]
Delineating associations of progressive pleuroparenchymal fibroelastosis in patients with pulmonary fibrosis
(European Respiratory Soc Journals Ltd, 2023) Gudmundsson, Eyjolfur; Zhao, An; Mogulkoc, Nesrin; van Beek, Frouke; Goos, Tinne; Brereton, Christopher J.; Veltkamp, Marcel
Background Computer quantification of baseline computed tomography (CT) radiological pleuroparenchymal fibroelastosis (PPFE) associates with mortality in idiopathic pulmonary fibrosis (IPF). We examined mortality associations of longitudinal change in computer-quantified PPFE-like lesions in IPF and fibrotic hypersensitivity pneumonitis (FHP). Methods Two CT scans 6-36 months apart were retrospectively examined in one IPF (n=414) and one FHP population (n=98). Annualised change in computerised upper-zone pleural surface area comprising radiological PPFE-like lesions (Delta-PPFE) was calculated.Delta-PPFE >1.25% defined progressive PPFE above scan noise. Mixed-effects models evaluated Delta-PPFE against change in visual CT interstitial lung disease (ILD) extent and annualised forced vital capacity (FVC) decline. Multivariable models were adjusted for age, sex, smoking history, baseline emphysema presence, antifibrotic use and diffusion capacity of the lung for carbon monoxide. Mortality analyses further adjusted for baseline presence of clinically important PPFE-like lesions and ILD change. Results Delta-PPFE associated weakly with ILD and FVC change. 22-26% of IPF and FHP cohorts demonstrated progressive PPFE-like lesions which independently associated with mortality in the IPF cohort (hazard ratio 1.25, 95% CI 1.16-1.34, p<0.0001) and the FHP cohort (hazard ratio 1.16, 95% CI 1.00-1.35, p=0.045). Interpretation Progression of PPFE-like lesions independently associates with mortality in IPF and FHP but does not associate strongly with measures of fibrosis progression.
Development and Validation of a Clinical Diagnostic Scoring System for the Diagnosis of Idiopathic Pulmonary Fibrosis
(Amer Thoracic Soc, 2021) Pastre, Jean; Barnett, Scott D.; Ksovreli, Inga; Mogulkoc, Nesrin; Ramalingam, Vijaya; Fukuda, Cesar; Yelisetty, Anusha
Rationale: Interpreting the radiologic data in conjunction with an objective clinical score could help to harmonize idiopathic pulmonary fibrosis (IPF) diagnosis and improve accuracy. Objectives: We sought to establish and validate a multivariable objective scoring model based on clinical parameters by stratifying the risk of patients having IPF diagnosed versus having other forms of interstitial lung disease (ILD) diagnosis. Methods: A clinical score was derived from review of patients evaluated at the Inova Fairfax ILD Program and validated in three distinct cohorts. On the basis of known IPF clinical characteristics, a multivariable model was created and assessed by using receiver operating characteristic curves. Results: There were 844 patients with ILD with either IPF (n = 347, 41%) or non-IPF ILD (n = 497, 59%) diagnosis. On the basis of calculated odds ratios, a score was assigned to each of the following clinical parameters: age, sex, smoking history, race or ethnicity, ILD family history, exposures, presence of connective tissue disease signs or symptoms, and velcro crackles. The final Fairfax IPF Clinical Score (FICS) ranged from 1 to 25. The clinical diagnostic score system was accurate in predicting IPF, as measured by the area under the curve (0.88) in the derivation cohort, with similar areas under the curve of 0.91, 0.81, and 0.71 being demonstrated in the respective validation cohorts. Conclusions: The FICS appears to be an accurate tool for estimating the pretest probability of IPF in patients with ILD. How the FICS performs in conjunction with the various high-resolution computed tomographic patterns remains to be determined. This model could ultimately be useful for increasing the degree of confidence in the final diagnosis and could help to obviate the need for lung biopsy in cases with non-usual interstitial pneumonia patterns on high-resolution computed tomographic images.
Differences between ANCA positive and negative lung fibrosis cases without vasculitis
(European Respiratory Soc Journals Ltd, 2019) Mogulkoc, Nesrin; Tas, Mehmet Nedim; Kabasakal, Yasemin; Bishop, Paul William; Littnerova, Simona; Hejduk, Karel; Vasakova, Martina
[No abstract available]
Differences in Baseline Characteristics and Access to Treatment of Newly Diagnosed Patients With IPF in the EMPIRE Countries
(Frontiers Media Sa, 2021) Kolonics-Farkas, Abigel Margit; Sterclova, Martina; Mogulkoc, Nesrin; Lewandowska, Katarzyna; Muller, Veronika; Hajkova, Marta; Kramer, Mordechai
Idiopathic pulmonary fibrosis (IPF) is a rare lung disease with poor prognosis. The diagnosis and treatment possibilities are dependent on the health systems of countries. Hence, comparison among countries is difficult due to data heterogeneity. Our aim was to analyse patients with IPF in Central and Eastern Europe using the uniform data from the European Multipartner IPF registry (EMPIRE), which at the time of analysis involved 10 countries. Newly diagnosed IPF patients (N = 2,492, between March 6, 2012 and May 12, 2020) from Czech Republic (N = 971, 39.0%), Turkey (N = 505, 20.3%), Poland (N = 285, 11.4%), Hungary (N = 216, 8.7%), Slovakia (N = 149, 6.0%), Israel (N = 120, 4.8%), Serbia (N = 95, 3.8%), Croatia (N = 87, 3.5%), Austria (N = 55, 2.2%), and Bulgaria (N = 9, 0.4%) were included, and Macedonia, while a member of the registry, was excluded from this analysis due to low number of cases (N = 5) at this timepoint. Baseline characteristics, smoking habit, comorbidities, lung function values, CO diffusion capacity, high-resolution CT (HRCT) pattern, and treatment data were analysed. Patients were significantly older in Austria than in the Czech Republic, Turkey, Hungary, Slovakia, Israel, and Serbia. Ever smokers were most common in Croatia (84.1%) and least frequent in Serbia (39.2%) and Slovakia (42.6%). The baseline forced vital capacity (FVC) was >80% in 44.6% of the patients, between 50 and 80% in 49.3%, and 80% were registered in Poland (63%), while the least in Israel (25%). A typical usual interstitial pneumonia (UIP) pattern was present in 67.6% of all patients, ranging from 43.5% (Austria) to 77.2% (Poland). The majority of patients received antifibrotic therapy (64.5%); 37.4% used pirfenidone (range 7.4-39.8% between countries); and 34.9% nintedanib (range 12.6-56.0% between countries) treatment. In 6.8% of the cases, a therapy switch was initiated between the 2 antifibrotic agents. Significant differences in IPF patient characteristics and access to antifibrotic therapies exist in EMPIRE countries, which needs further investigation and strategies to improve and harmonize patient care and therapy availability in this region.
Differences in baseline characteristics of newly diagnosed IPF patents in the EMPIRE countries
(European Respiratory Soc Journals Ltd, 2018) Mueller, Veronika; Sterclova, Martina; Mogulkoc, Nesrin; Kus, Jan; Hajkova, Marta; Jovanovic, Dragana; Tekavec-Trkanjec, Jasna; Svoboda, Michal; Janotova, Marketa; Vasakova, Martina
Does body mass index have prognostic significance for patients with idiopathic pulmonary fibrosis?
(European Respiratory Soc Journals Ltd, 2018) Mogulkoc, Nesrin; Sterclova, Martina; Mueller, Veronika; Kus, Jan; Hajkova, Marta; Jovanovic, Dragana; Tekavec-Trkanjec, Jasna; Janotova, Marketa; Vasakova, Martina
Does body mass index influence survival of patients with idiopathic pulmonary fibrosis?
(European Respiratory Soc Journals Ltd, 2018) Mogulkoc, Nesrin; Uysaler, Burcu; Bishop, Paul
Dynamic computed tomography in solitary pulmonary nodules
(Lippincott Williams & Wilkins, 2008) Bayraktaroglu, Selen; Savas, Recep; Basoglu, Oezen Kacmaz; Cakan, Alparslan; Mogulkoc, Nesrin; Cagirici, Ufuk; Alper, Huedaver
Objective: To evaluate the utility of dynamic computed tomography (CT) imaging in the management of solitary pulmonary nodules. Methods: We examined solitary pulmonary nodules in 45 patients. The nodules included in the study were solid, homogenous, and spherical or oval-shaped in precontrast images. Nodules that had calcification or fat density were excluded from the study. We were not able to obtain clinical or pathological diagnosis of the nodules in 23 patients, and we excluded these patients from statistical analysis. Patients were examined with 2-mm collimation and I-mm reconstruction interval. Computed tomography examinations were done before and after injection of contrast material. Computed tomography scans of the nodule were obtained in the first, second, third, fourth, and fifth minutes after contrast injection. Results: The malignancy prevalence was 40%. The mean enhancement of malignant nodules were significantly higher than the benign ones. Also, the peak attenuation value obtained after the administration of contrast material was 82.44 +/- 19.56 HU in malignant lesions and 54 +/- 23.10 HU in benign ones, with statistical significance (P = 0.006). Using enhancement values greater than 15 HU as a threshold for malignancy, the calculated sensitivity, specificity, positive and negative predictive values, and accuracy of the dynamic CT examination were 100%, 69.2%, 69.2%, 100%, and 81%, respectively. Conclusion: Dynamic CT imaging demonstrates significantly greater enhancement in malignant nodules than in benign ones. Lung nodule enhancement of 15 HU or less strongly indicates benignity.
The effect of metformin on clinically relevant outcomes in 3,144 IPF patients from the EMPIRE registry
(European Respiratory Soc Journals Ltd, 2020) Jovanovic, Dragana; Sterclova, Martina; Mogulkoc, Nesrin; Lewandowsk, Katarzyna; Muller, Veronika; Hajkova, Marta; Kaeva, Biserka Jovovska
[No Abstract Available]
Effects of cardiopulmonary rehabilitation on pulmonary arterial hypertension: A prospective, randomized study
(Baycinar Medical Publ-Baycinar Tibbi Yayincilik, 2019) Karapolat, Hale; Cinar, Muyesser Ece; Tanigor, Goksel; Nalbantgil, Sanem; Kayikcioglu, Meral; Mogulkoc, Nesrin; Kultursay, Hakan
Objectives: This study aims to investigate the effects of cardiopulmonary rehabilitation (CPR) on cardiopulmonary function, quality of life, depression, and hemodynamic parameters in patients with pulmonary arterial hypertension (PAH) and to compare the efficacy of hospital- and home-based exercise programs. Patients and methods: Between December 2014 to May 2016, a total of 30 patients with PAH were included in the study. the patients were randomly assigned to either a hospital-based (n=15) or home-based exercise program (n=15). the hospital group was assigned to a cardiac rehabilitation (three days/week, 1 hour/week 50 to 70% maximal oxygen uptake [PvO(2)]) program for eight weeks. the home-based exercise group received home exercises alone. Before and after eight weeks of rehabilitation, all patients were evaluated for their functional status (PvO(2)), pulmonary function including forced expiratory volume in one sec (FEV1), mL, forced vital capacity (FVC), mL and FEV1/FVC%, quality of life using Short Form-36 (SF-36), depression severity using Beck Depression Inventory, and hemodynamic parameters including left ventricular ejection fraction, right ventricular ejection fraction, left ventricular end-systolic diameter, left ventricular end-diastolic diameter, and systolic pulmonary arterial pressure. Results: of the patients, 12 completed the study in each group. There was no statistically significant difference in the functional status, quality of life, depression severity, and hemodynamic parameters after the rehabilitation compared baseline between the groups. Conclusion: Based on our study results, short-term CPR seems not to be beneficial in patients with PAH. We recommend long-term rehabilitation programs to achieve more benefits from aerobic exercise training in this patient population.
The European MultiPartner IPF registry (EMPIRE): validating long-term prognostic factors in idiopathic pulmonary fibrosis
(Bmc, 2020) Tran, Tanja; Sterclova, Martina; Mogulkoc, Nesrin; Lewandowska, Katarzyna; Muller, Veronika; Hajkova, Marta; Martusewia-Boros, Magdalena
Background Several registries of idiopathic pulmonary fibrosis (IPF) have been established to better understand its natural history, though their size and duration of follow-up are limited. Here, we describe the large European MultiPartner IPF Registry (EMPIRE) and validate predictors of long-term survival in IPF. Methods the multinational prospective EMPIRE registry enrolled IPF patients from 48 sites in 10 Central and Eastern European countries since 2014. Survival from IPF diagnosis until death was estimated, accounting for left-truncation. the Cox proportional hazards regression model was used to estimate adjusted hazard ratios (HR) of death for prognostic factors, using restricted cubic splines to fit continuous factors. Results the cohort included 1620 patients (mean age at diagnosis 67.6 years, 71% male, 63% smoking history), including 75% enrolled within 6 months of diagnosis. Median survival was 4.5 years, with 45% surviving 5 years post-diagnosis. Compared with GAP stage I, mortality was higher with GAP stages II (HR 2.9; 95% CI: 2.3-3.7) and III (HR 4.0; 95% CI: 2.8-5.7) while, with redefined cut-offs, the corresponding HRs were 2.7 (95% CI: 1.8-4.0) and 5.8 (95% CI: 4.0-8.3) respectively. Mortality was higher with concurrent pulmonary hypertension (HR 2.0; 95% CI: 1.5-2.9) and lung cancer (HR 2.6; 95% CI: 1.3-4.9). Conclusions EMPIRE, one of the largest long-term registries of patients with IPF, provides a more accurate confirmation of prognostic factors and co-morbidities on longer term five-year mortality. It also suggests that some fine-tuning of the indices for mortality may provide a more accurate long-term prognostic profile for these patients.

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