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Does early diagnosis of idiopathic pulmonary fibrosis matter? Real- world´s data from the EMPIRE registry

Martina Vašáková, Martina Sterclova, Jan Kus, Vladimir Bartos, Marta Hajkova, Martina Doubkova, Veronika Muller, Martina Plackova, Monika Zurkova, Vladimira Lostakova, Ladislav Lacina, Vladimir Rihak, Frantisek Petrik, Pavlina Lisá, Radka Bittenglova, Dragana Jovanovic, Richard Tyl, Gustav Ondrejka, Hana Suldova, Jaroslav Lnenicka, Jana Psikalova, Tomas Snizek, Jiri Homolka, Renata Kralova, Jan Kervitzer, Michal Svoboda, Jana Strenkova
European Respiratory Journal 2016 48: PA794; DOI: 10.1183/13993003.congress-2016.PA794
Martina Vašáková
1Department of Respiratory Diseases of the 1st Medical School Charles University, Thomayer Hospital, Prague, Czech Republic
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Martina Sterclova
1Department of Respiratory Diseases of the 1st Medical School Charles University, Thomayer Hospital, Prague, Czech Republic
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Jan Kus
21st Department of Pulmonary Diseases, Institute of Tuberculosis and Lung Diseases, Warsaw, Poland
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Vladimir Bartos
3Pulmonary Department, University Hospital and Medical Faculty of Charles University in Hradec Kralove, Hradec Králové, Czech Republic
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Marta Hajkova
4Klinika Pneumologie a Ftizeologie, UN Bratislava, Bratislava, Slovakia
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Martina Doubkova
5Department of Pneumology and Phtiseology, Faculty of Medicine and University Hospital, Brno, Czech Republic
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Veronika Muller
6Department of Pulmonology, Semmelweis University, Budapest, Hungary
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Martina Plackova
7Department of Pneumology, Faculty Hospital Ostrava, Ostrava, Czech Republic
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Monika Zurkova
8Department of Respiratory Medicine, Faculty of Medicine and Dentistry, Palacky University and University Hospital, Olomouc, Czech Republic
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Vladimira Lostakova
8Department of Respiratory Medicine, Faculty of Medicine and Dentistry, Palacky University and University Hospital, Olomouc, Czech Republic
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Ladislav Lacina
9Clinic of Pneumology and Thoracic Surgery, Hospital Na Bulovce, Prague, Czech Republic
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Vladimir Rihak
10Department of Respiratory Diseases, Thomas Bata Regional Hospital Zlin, Zlin, Czech Republic
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Frantisek Petrik
11Department of Pneumology, 2nd Faculty of Medicine, Charles University in Prague and Motol University Hospital, Prague, Czech Republic
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Pavlina Lisá
11Department of Pneumology, 2nd Faculty of Medicine, Charles University in Prague and Motol University Hospital, Prague, Czech Republic
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Radka Bittenglova
12Departmant of Respiratory Diseases, Charles University in Plzen, Pilsen, Czech Republic
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Dragana Jovanovic
13Thoracic Oncology and ILD Dept., Clinical Centre of Serbia, Belgrade, Czech Republic
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Richard Tyl
14Department of Respiratory Diseases, Hospital in Novy Jicin, Novy Jicin, Czech Republic
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Gustav Ondrejka
14Department of Respiratory Diseases, Hospital in Novy Jicin, Novy Jicin, Czech Republic
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Hana Suldova
15Pulmonary Department, Hospital Ceské Budejovice, Ceské Budejovice, Czech Republic
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Jaroslav Lnenicka
16Department of Pulmonary Diseases and Tuberculosis, Masaryk Hospital, Usti nad Labem, Czech Republic
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Jana Psikalova
17PneumoAllergolog Department, Kromeriz Hospital, Kromeriz, Czech Republic
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Tomas Snizek
18Department of Respiratory Diseases, Hospital Jihlava, Jihlava, Czech Republic
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Jiri Homolka
191st Clinic of Tuberculosis and Respiratory Diseases, 1st Medical School, Charles University, Prague, Czech Republic
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Renata Kralova
20Department of Pulmonology, Regional Hospital Pardubice, Pardubice, Czech Republic
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Jan Kervitzer
21Department of Respiratory Diseases, Hospital Znojmo, Znojmo, Czech Republic
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Michal Svoboda
22Institute of Biostatistics and Analyses, Masaryk University, Brno, Czech Republic
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Jana Strenkova
22Institute of Biostatistics and Analyses, Masaryk University, Brno, Czech Republic
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Abstract

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.

METHODS: The study was retrospective using data from EMPIRE registry (European Multipartner IPF registry). 566 patients were stratified according to duration of symptoms preceding the diagnosis of IPF: in earlier diagnosed group (ED) the diagnosis was stated within 1 year from onset of symptoms (383 patients), and in the later diagnosed (LD) group symptoms lasted longer than 1 year (183 patients). The survival from the diagnosis up to 84 months was evaluated by Kaplan-Meier curve and statistical significance tested by Log Rank test. The vital capacity (VC) initial values and VC decline in 6, 12, and 18 months of follow-up were evaluated and compared between both groups using t-test. P value less than 0.05 was considered statistically significant.

RESULTS: The Kaplan-Meier survival curves showed better survival in ED compared to LD patients. The median survival in ED was 63.1 and in the LD 43.9 months (p=0.018). VC at the time of diagnosis was higher in ED (82.9% of PV in ED vs 75.8% of PV in LD; p=0.008). There was no difference in rate of VC decline.

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.

The study was supported by grant NT 13433-4/2012, IGA MZCR.

  • Idiopathic pulmonary fibrosis
  • Longitudinal study
  • Copyright ©the authors 2016
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Does early diagnosis of idiopathic pulmonary fibrosis matter? Real- world´s data from the EMPIRE registry
Martina Vašáková, Martina Sterclova, Jan Kus, Vladimir Bartos, Marta Hajkova, Martina Doubkova, Veronika Muller, Martina Plackova, Monika Zurkova, Vladimira Lostakova, Ladislav Lacina, Vladimir Rihak, Frantisek Petrik, Pavlina Lisá, Radka Bittenglova, Dragana Jovanovic, Richard Tyl, Gustav Ondrejka, Hana Suldova, Jaroslav Lnenicka, Jana Psikalova, Tomas Snizek, Jiri Homolka, Renata Kralova, Jan Kervitzer, Michal Svoboda, Jana Strenkova
European Respiratory Journal Sep 2016, 48 (suppl 60) PA794; DOI: 10.1183/13993003.congress-2016.PA794

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Does early diagnosis of idiopathic pulmonary fibrosis matter? Real- world´s data from the EMPIRE registry
Martina Vašáková, Martina Sterclova, Jan Kus, Vladimir Bartos, Marta Hajkova, Martina Doubkova, Veronika Muller, Martina Plackova, Monika Zurkova, Vladimira Lostakova, Ladislav Lacina, Vladimir Rihak, Frantisek Petrik, Pavlina Lisá, Radka Bittenglova, Dragana Jovanovic, Richard Tyl, Gustav Ondrejka, Hana Suldova, Jaroslav Lnenicka, Jana Psikalova, Tomas Snizek, Jiri Homolka, Renata Kralova, Jan Kervitzer, Michal Svoboda, Jana Strenkova
European Respiratory Journal Sep 2016, 48 (suppl 60) PA794; DOI: 10.1183/13993003.congress-2016.PA794
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