Pneumologie 2019; 73(S 01)
DOI: 10.1055/s-0039-1678278
Posterbegehung (P22) – Sektion Klinische Pneumologie
Fortschritte bei ILD
Georg Thieme Verlag KG Stuttgart · New York

Outcome differences between idiopathic pulmonary fibrosis (IPF) and other interstitial lung diseases (ILD) – data from the EXCITING registry

M Kreuter
1   Zentrum für Interstitielle und Seltene Lungenerkrankungen, Pneumologie und Beatmungsmedizin, Thoraxklinik, Universitätsklinikum Heidelberg und Translationales Zentrum für Lungenforschung Heidelberg (Tlrc), Mitglied des Deutschen Zentrums für Lungenforschung (Dzl)
,
HJ Kabitz
2   Pneumology, Klinikum Konstanz, Germany
,
L Hagmeyer
3   Hospital Bethanien Solingen, Clinic of Pneumology and Allergology, Center of Sleep Medicine and Respiratory Care, Germany
,
P Hammerl
4   Chest Clinic Immenhausen, Immenhausen, Germany
,
A Esselmann
5   Outpatient Center for Pulmonology, Warendorf, Germany
,
C Wiederhold
6   Outpatient Center for Pulmonology, Fulda, Germany
,
D Skowasch
7   Medical Clinic II, University Hospital Bonn, Germany
,
C Stolpe
8   Outpatient Center for Pulmonology, Ibbenbüren, Germany
,
M Joest
9   Malteser Center for Pulmonology and Allergology, Bonn
,
S Veitshans
10   Outpatient Center for Pulmonology, Böblingen, Germany
,
S Witt
11   Institute of Health Economics and Healthcare Management, Helmholtz Centre Munich GmbH, German Research Centre for Environmental Health, Member of the German Centre for Lung Research (Dzl), Comprehensive Pneumology Centre Munich (Cpcm), Germany
,
R Leidl
12   Institute of Health Economics and Healthcare Management, Helmholtz Centre Munich GmbH, German Research Centre for Environmental Health, Member of the German Centre for Lung Research (Dzl), Comprehensive Pneumology Centre Munich, Munich Centre of Health Sciences, Ludwig Maximilian University Munich, Germany
,
A Hellmann
13   Outpatient Center for Pulmonology, Augsburg, Germany
,
M Pfeifer
14   Medical Clinic II, University of Regensburg and Klinikum Donaustauf, Germany
,
J Behr
15   Medical Clinic V, University Clinic, Ludwig-Maximilians-Universitymunich, Comprehensive Pneumology Centremunich (Cpc-M), Member of the German Centre for Lung Research (Dzl), Germany
,
D Kauschka
16   Patient Support Group Lungenfibrose e. V., Essen, Germany
,
A Günther
17   Medical Clinic II, University Hospital Giessen, Universities of Giessen and Marburg Lung Centre (Ugmlc), Member of the German Centre for Lung Research (Dzl),
,
FJF Herth
18   Centre for Interstitial and Rare Lung Diseases, Pneumology and Respiratory Critical Care Medicine, Thoraxklinik, University of Heidelberg, Translational Lung Research Centre Heidelberg (Tlrch), German Centre for Lung Research (Dzl), Germany
,
P Markart
19   Medical Clinic II, University Hospital Giessen, Universities of Giessen and Marburg Lung Centre (Ugmlc), Member of the German Centre for Lung Research (Dzl), Medical Clinic V (Pneumology), Cardiothoracic Centre, Campus Fulda, University Medicine Marburg, Germany
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Publikationsdatum:
19. Februar 2019 (online)

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Background Available epidemiological data suggest that IPF is a frequent and unfavourable fibrosing ILD. Yet, data on outcome differences between IPF and other ILDs are scant.

Method The prospective, multi-centre German ILD registry “EXCITING” was analysed for differences in outcomes between IPF and other ILDs.

Results Out of 601 patients (60% male, 53% ex-/smokers, medians: age 64 years, FVC 75%, DLCO 53%), 151 (25%) suffered from IPF (76% male, 58% ex-/smokers, medians: age 74 years, FVC 69%, DLCO 39%). Of 314 all-cause hospitalisations 32% occurred in IPF. Also, IPF accounted for 29% and 35% of respiratory and acute exacerbation (AE) related hospitalisations, respectively. Out of 91 deceased patients, 46% had IPF. Time to death was 101 months for IPF compared to 229 months for non-IPF ILDs (non-IPF, p < 0.001). 91% of death followed hospitalisations, 50% of death occurred after respiratory related hospitalisations, of which were 36% in IPF. Mortality after AE-hospitalisation was similar for IPF and non-IPF (45%). Yet, for respiratory related hospitalisation without AE, mortality was higher for IPF (50%) compared to non-IPF (15%). Progression free survival (PFS, time from diagnosis to decrease in FVC > 10%, hospitalization due to respiratory reason or death), differed between IPF vs. non-IPF with a mean of 62 months vs. 113 months (p = 0.006). In IPF, there was also a trend for differences in PFS related to baseline FVC: for FVC < 50% 52 months, FVC 50 – 90% 63 months and for FVC > 90% 107 months (p = 0.077).

Conclusions IPF is a common ILD subtype with frequent hospitalisations, more rapid progression and worse mortality than other ILDs.