Az előadás megjelenését a Boehringer Ingelheim támogatta. Az előadás tartalma az előadó (eredményeit és) önálló szakmai álláspontját tükrözi és nem tekinthető a Boehringer Ingelheim részéről közzétett szakmai, vagy egyéb tájékoztatásnak vagy állásfoglalásnak. A megemlített termékek használatakor az érvényes alkalmazási előírás az irányadó. Lezárás dátuma: 2017.10.25.

HU/OFE/1017/00036

EMPIRE regiszterhazai és nemzetközi tapasztalatok

Prof. Dr. Müller VeronikaSemmelweis Egyetem Pulmonológiai Klinika

IPF Centrumok

Miskolc

Debrecen

SzegedPécs

Semmelweis Egyetem

GyőrTörökbálint

Székesfehérvár

OKPI

TUKEB engedély kiterjesztése

Summary report of EMPIRE registryHUNGARY (N=96)

Data export: 22 December 2016

MTT: Müller Veronika, Bohács Anikó, Zsiray Miklós, Szalai Zsuzsanna, Balikó Zoltán, Somfay Attila és

Szilasi Mária

Selection of data set for analysis

Patients in EMPIRE registryn=1405

Patients included to analysis n=1384

Patients excluded from analysis

n=21

No information about date of diagnosis, change of diagnosis

2016.12.22

n=1384

Representation of countries in EMPIRE registry

N (%)

Czech Republic 669 (48.3%)

Poland 241 (17.4%)

Turkey 217 (15.7%)

Slovakia 115 (8.3%)

Hungary 96 (6.9%)

Serbia 31 (2.2%)

Croatia 15 (1.1%)

>500 100–500 50–100 <50

2016.12.22

n=96

before 2005 2005 2006 2007 2008 2009 2010 2011 2012 2013 2014 2015 20160

20

40

60

80

100

120

1 1 3 5 5 8 913

2025

40

73

96

Year of diagnosis

Cum

ula

tive

num

ber

of

patients

Number of newly diagnosed patients

2016.12.22

European Respiratory Society Congress – ERS Congress 2017

9-13. September 2017, Milan

Comorbidity in IPF patientsEMPIRE registry

(European Multipartner IPF registry)

Dragana Jovanovic,Nesrin Mogulkoc, Martina Šterclová, Beata Zolnowska, Vladimír Bartoš, Martina Plačková, Veronika Müller, Ladislav Lacina, Robert Slivka, Martina Doubková, Marina Roksandić Milenkovic, Radka Bittenglová, Imrich Jonner, Magdalena Martusewicz-Boros, Monika Žurková, Ilona Binková, Małgorzata Sobiecka, Richard Tyl, Marzena Trzaska-Sobczak, Pawel Sliwinski, Vladimíra Lošťáková, Sebastian Majewski, Pavlína Lisá, Katarzyna Lewandowska, Hana Šuldová, Jasna Tekavec-Trkanjec, Amelia Szymanowska-Narloch, Lenka Šišková, Anikó Bohács, Ján Plutinský, Bohumil Matula, Štefan Tóth, František Petřík, Margita Bučeková, Jana Pšíkalová, Miklós Zsiray, Jaroslav Lněnička, Vladimír Řihák, Zsuzsanna Szalai, Zoltán Balikó, Štefan Laššán, Pavlína Musilová, Imre Lajkó, Tomáš Snížek, Jan Anton, Jiří Homolka, Peter Palúch, Róbert Vyšehradský, Renata Králová, Jan Kervitzer, Daniel Doležal, Maria Szilasi, Jan Kus, Marta Hájková, Michal Svoboda, Jana Strenková, Martina Vašáková

Selection of data set for analysis

Patients in EMPIRE registryN=1229

Patients included to analysis N=1210

Patients excluded from analysis

N=19

No information about date of diagnosis, change of

diagnosis

2016. November

N=1210

Representation of countries in EMPIRE registry

N (%)

Czech Republic

640 (52.9%)

Poland 213 (17.6%)

Turkey 141 (11.7%)

Slovakia 106 (8.8%)

Hungary 80 (6.6%)

Serbia 30 (2.5%)

Determine baseline clinical characteristics to predict rapid decline of lung function in IPF patients included into the EMPIRE registry.

Introduction •The rate of change in FVC or TLco among IPF patients is highly variable, with high proportion of patients showing increased rates of decline in FVC (56%) or TLco (34%) over a 6 months period. • No baseline clinical characteristics could be identified in these selected 324 IPF patients which can predict rapid decline using the data of EMPIRE registry.

Conclusions

Aim

Methods

European MultiPartner IPF REgistry: http://empire.registry.cz/index-en.php

Results

Can clinical data predict rapid decline of lung function in idiopathic pulmonary fibrosis (IPF) patients?

Key feature of IPF is decline in forced vital capacity (FVC) and CO diffusion (TLco), 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 registry in the CEE region including patients from Czech Republic, Hungary, Poland, Slovakia, Serbia and Turkey. Out of 1229 patients included into the registry until October 2016 a total of 324 patients with available data on FVC and TLco at inclusion and at 6 months rapid decliners (RD: FVC >10% or TLco >10% in 6 months) and controls (C) were included into the analysis. Patients’ age, HRCT pattern, smoking behavior, dyspnea, comorbidities were analyzed between groups to find clinical determinants of RD of FVC and/or TLco.

References 1. Travis WD, Costabel U, Hansell DM, King TE Jr, Lynch DA, Nicholson AG, Ryerson CJ, Ryu JH, Selman M, Wells AU, Behr J, Bouros D, Brown KK, Colby TV, Collard HR, Cordeiro CR, Cottin V, Crestani B, Drent M, Dudden RF, Egan J, Flaherty K, Hogaboam C, Inoue Y, Johkoh T, Kim DS, Kitaichi M, Loyd J, Martinez FJ, Myers J, Protzko S, Raghu G, Richeldi L, Sverzellati N, Swigris J, Valeyre D; ATS/ERS Committee on Idiopathic Interstitial Pneumonias. An official American Thoracic Society/European Respiratory Society statement: Update of the international multidisciplinary classification of the idiopathic interstitial pneumonias. Am J Respir Crit Care Med. 2013 Sep 15;188(6):733-48.2. Kärkkäinen M, Kettunen HP, Nurmi H, Selander T, Purokivi M, Kaarteenaho R. Effect of smoking and comorbidities on survival in idiopathic pulmonary fibrosis. Respir Res. 2017 Aug 22;18(1):160. 3. Kreuter M, Ehlers-Tenenbaum S, Palmowski K, Bruhwyler J, Oltmanns U, Muley T, Heussel CP, Warth A, Kolb M, Herth FJ. Impact of Comorbidities on Mortality in Patients with Idiopathic Pulmonary Fibrosis. PLoS One. 2016 Mar 29;11(3):e0151425.4. Collard HR, King TE Jr, Bartelson BB, Vourlekis JS, Schwarz MI, Brown KK. Changes in clinical and physiologic variables predict survival in idiopathic pulmonary fibrosis. Am J Respir Crit Care Med. 2003 Sep 1;168(5):538-42.5. Salisbury ML, Xia M, Zhou Y, Murray S, Tayob N, Brown KK, Wells AU, SchmidtSL, Martinez FJ, Flaherty KR. Idiopathic Pulmonary Fibrosis: Gender-Age-Physiology Index Stage for Predicting Future Lung Function Decline. Chest. 2016 Feb;149(2):491-8. 6. Behr J. Disease Progression in Idiopathic Pulmonary Fibrosis - FVC is Not Enough. Am J Respir Crit Care Med. 2017 Jul 12. doi: 10.1164/rccm.7. Ley B, Bradford WZ, Vittinghoff E, Weycker D, du Bois RM, Collard HR. Predictors of Mortality Poorly Predict Common Measures of Disease Progression in Idiopathic Pulmonary Fibrosis. Am J Respir Crit Care Med. 2016 Sep 15;194(6):711-8.8. Khadawardi H, Mura M. A simple dyspnoea scale as part of the assessment to predict outcome across chronic interstitial lung disease. Respirology. 2017 Apr;22(3):501-507.

Veronika Müller1, Nesrin Mogulkoc2, Martina Šterclová3, Beata Zolnowska4, Vladimír Bartoš5, Martina Plačková6, Ladislav Lacina7, Robert Slivka8, Martina Doubková9, Marina Roksandić Milenkovic10, Radka Bittenglová11, Imrich Jonner8, Magdalena Martusewicz-Boros12, Monika Žurková13, Ilona Binková9, Małgorzata Sobiecka4, Richard Tyl14, Marzena Trzaska-Sobczak15, Pawel Sliwinski16, Vladimíra Lošťáková13, Sebastian Majewski17, Pavlína Lisá18, Katarzyna Lewandowska3, Hana Šuldová19, Jasna Tekavec-Trkanjec20, Amelia Szymanowska-

Narloch21, Lenka Šišková22, Anikó Bohács1, Ján Plutinský23, Bohumil Matula24, Štefan Tóth25, František Petřík18, Margita Bučeková8, Jana Pšíkalová26, Miklós Zsiray27, Jaroslav Lněnička28, Vladimír Řihák22, Zsuzsanna Szalai29, Zoltán Balikó30, Štefan Laššán31, Pavlína Musilová32, Imre Lajkó33, Tomáš Snížek32, Jan Anton2, Jiří Homolka34, Peter Palúch2, Róbert Vyšehradský35, Renata Králová36, Jan Kervitzer37, Daniel Doležal28, Maria Szilasi38, Jan Kus3, Dragana Jovanovic10, Marta Hájková31, Michal Svoboda39, Jana Strenková39, Martina Vašáková3

1 Department of Pulmonology, Semmelweis University, Budapest, Hungary; 2 Department of Pulmonary Medicine, Ege University Medical School, Izmir, Turkey; 3 Department of Respiratory Diseases of the 1st Medical School Charles University, Thomayer Hospital, Prague, Czech Republic; 4 1st Department of Pulmonary Diseases, Institute of Tuberculosis and Lung Diseases, Warsaw, Poland; 5 Pulmonary Department, University Hospital and Medical Faculty of Charles University in Hradec Kralove, Hradec Králové, Czech Republic; 6 Department of Pneumology, Faculty Hospital Ostrava, Ostrava, Czech Republic; 7 Clinic of Pneumology and Thoracic Surgery, Hospital Na Bulovce, Prague, Czech Republic; 8 National Institute of Tuberculosis, Lung Disorders and Thoracic Surgery Vyšné Hagy, Slovakia; 9 Department of Pneumology, Faculty of Medicine and University Hospital, Brno, Czech Republic; 10 University Hospital of Pulmonology, Clinical Center of Serbia, Belgrade, Serbia; 11 Department of Respiratory Diseases, Charles University in Plzen, Pilsen, Czech Republic; 12 3rd Department of Pulmonary Diseases, Institute of Tuberculosis and Lung Diseases, Warsaw, Poland; 13 Department of Respiratory Medicine, Faculty of Medicine and Dentistry, Palacky University and University Hospital, Olomouc, Czech Republic; 14

Department of Respiratory Diseases, Hospital in Novy Jicin, Novy Jicin, Czech Republic; 15 Department of Pneumology, Medical University of Silesia in Katowice, Poland; 16 4th Department of Pulmonary Diseases, Institute of Tuberculosis and Lung Diseases, Warsaw, Poland; 17 Department of Pneumology and Allergy, Medical University of Lodz, Lodz, Poland; 18 Department of Pneumology, 2nd Faculty of Medicine, Charles University in Prague and Motol University Hospital, Prague, Czech Republic; 19 Pulmonary Department, Hospital Ceske Budejovice, Ceske Budejovice, Czech Republic; 20 Pulmonary Department, University Hospital Dubrava, Zagreb, Croatia; 21 Department of Allergology and Pneumology, Medical University of Gdansk, Gdansk, Poland; 22 Department of Respiratory Diseases, Thomas Bata Regional Hospital Zlin, Zlin, Czech Republic; 23 Pneumology and Phthisiology Outpatient Centre, ZAPA JJ Ltd., Levice, Slovakia; 24 Pneumology and Phthisiology, Specialized Hospital of St. Zoerardus Zobor, Nitra, Slovakia; 25 Clinic of Pneumology and Phthisiology, L. Pasteur University Hospital Kosice, Kosice, Slovakia; 26 Pneumology and Allergology Department, Kromeriz Hospital, Kromeriz, Czech Republic; 27 Department of Pulmonology, National Korányi Tuberculosis and Pulmonology Institute, Budapest, Hungary; 28 Department of Pulmonary Diseases and Tuberculosis, Masaryk Hospital, Usti nad Labem, Czech Republic; 29 Department of Pulmonology, Petz Aladár County Teaching Hospital, Gyor, Hungary; 30 Department of Pulmonology, Faculty of Medicine, University of Pecs, Pecs, Hungary; 31 Clinic of Pneumology and Phthisiology, University Hospital Bratislava, Bratislava, Slovakia; 32 Department of Respiratory Diseases,

Hospital Jihlava, Jihlava, Czech Republic; 33 Department of Pulmonology, University of Szeged, Szeged, Hungary; 34 1st Clinic of Tuberculosis and Respiratory Diseases, 1st Medical School, Charles University, Prague, Czech Republic; 35 Clinic of Pneumology and Phthisiology, University Hospital Martin, Martin, Slovakia; 36 Department of Pulmonology, Regional Hospital Pardubice, Pardubice, Czech Republic; 37 Department of Respiratory Diseases, Hospital Znojmo, Znojmo, Czech Republic; 38 Centre for Pulmonology, University of Debrecen, Clinical Centre, Debrecen, Hungary; 39 Institute of Biostatistics and Analyses, Masaryk University, Brno, Czech Republic

Figure 6. Decline of FVC in relation to other characteristics

Rapid decline of FVCP-value

No (N=183) Yes (N=93)

SexWomen 57 (31.1%) 35 (37.6%)

0.284Men 126 (68.9%) 58 (62.4%)

Age at diagnosis 67.5 (54.4–82.9) 67.6 (48.3–82.8) 0.583

HRCTTypical 139 (76.0%) 72 (77.4%)

0.881Atypical 44 (24.0%) 21 (22.6%)

SmokingNon-smoker 89 (48.6%) 51 (54.8%)

0.598Ex-smoker 4 (2.2%) 2 (2.2%)Smoker 90 (49.2%) 40 (43.0%)

NYHA

I 4 (2.5%) 0 (0.0%)

0.498II 108 (68.4%) 59 (67.8%)III 45 (28.5%) 27 (31.0%)IV 1 (0.6%) 1 (1.1%)

Comorbities

Arterial hypertension 93 (50.8%) 48 (51.6%) 0.999Coronary heart disease 39 (21.3%) 25 (26.9%) 0.365Diabetes mellitus 38 (20.8%) 17 (18.3%) 0.750Hyperlipidaemia 35 (19.1%) 22 (23.7%) 0.432Osteoporosis 34 (18.6%) 15 (16.1%) 0.739GERD 24 (13.1%) 17 (18.3%) 0.284Arrhythmias 17 (9.3%) 11 (11.8%) 0.531

Categorical parameters are described by absolute (relative) frequencies and tested by Fisher exact test.Continuous parameter is described by median (5th–95th percentile) and tested by Mann-Whitney test.

Figure 7. Decline of TLCO in relation to other characteristics

Rapid decline of TLCO P-valueNo (N=113) Yes (N=129)

SexWomen 37 (32.7%) 46 (35.7%)

0.685Men 76 (67.3%) 83 (64.3%)

Age at diagnosis 67.7 (55.6–82.5) 66.6 (49.5–82.9) 0.069

HRCTTypical 88 (77.9%) 101 (78.3%)

0.999Atypical 25 (22.1%) 28 (21.7%)

SmokingNon-smoker 53 (46.9%) 70 (54.3%)

0.433Ex-smoker 1 (0.9%) 2 (1.6%)Smoker 59 (52.2%) 57 (44.2%)

NYHA

I 3 (3.1%) 0 (0.0%)

0.193II 68 (70.1%) 80 (67.2%)III 25 (25.8%) 38 (31.9%)IV 1 (1.0%) 1 (0.8%)

Comorbities

Arterial hypertension 62 (54.9%) 65 (50.4%) 0.520Coronary heart disease 25 (22.1%) 28 (21.7%) 0.999Diabetes mellitus 23 (20.4%) 23 (17.8%) 0.627Hyperlipidaemia 21 (18.6%) 32 (24.8%) 0.277Osteoporosis 19 (16.8%) 24 (18.6%) 0.739GERD 13 (11.5%) 23 (17.8%) 0.206Arrhythmias 17 (15.0%) 10 (7.8%) 0.101

Categorical parameters are described by absolute (relative) frequencies and tested by Fisher exact test.Continuous parameter is described by median (5th–95th percentile) and tested by Mann-Whitney test.

Most hol tartunk?

2017.10.19

A legnagyobb IPF regiszter

2017.10.19

1983 beteg5. Legnagyobb a SE centrum