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.jpg): Failed to open stream: No such file or directory in C:\Inetpub\vhosts\kidney.de\httpdocs\pget.php on line 117 Respir+Res
2018 ; 19
(1
): 141
Nephropedia Template TP
gab.com Text
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The European IPF registry (eurIPFreg): baseline characteristics and survival of
patients with idiopathic pulmonary fibrosis
#MMPMID30055613
Guenther A
; Krauss E
; Tello S
; Wagner J
; Paul B
; Kuhn S
; Maurer O
; Heinemann S
; Costabel U
; Barbero MAN
; Müller V
; Bonniaud P
; Vancheri C
; Wells A
; Vasakova M
; Pesci A
; Sofia M
; Klepetko W
; Seeger W
; Drakopanagiotakis F
; Crestani B
Respir Res
2018[Jul]; 19
(1
): 141
PMID30055613
show ga
BACKGROUND: Since 2009, IPF patients across Europe are recruited into the
eurIPFreg, providing epidemiological data and biomaterials for translational
research. METHODS: The registry data are based on patient and physician baseline
and follow-up questionnaires, comprising 1700 parameters. The mid- to long-term
objectives of the registry are to provide clues for a better understanding of IPF
phenotype sub-clusters, triggering factors and aggravating conditions, regional
and environmental characteristics, and of disease behavior and management.
RESULTS: This paper describes baseline data of 525 IPF subjects recruited from
11/2009 until 10/2016. IPF patients had a mean age of 68.1 years, and seeked
medical advice due to insidious dyspnea (90.1%), fatigue (69.2%), and dry
coughing (53.2%). A surgical lung biopsy was performed in 32% in 2009, but in
only 8% of the cases in 2016, possibly due to increased numbers of cryobiopsy. At
the time of inclusion in the eurIPFreg, FVC was 68.4% ± 22.6% of predicted value,
DLco ranged at 42.1% ± 17.8% of predicted value (mean value ± SD). Signs of
pulmonary hypertension were found in 16.8%. Steroids, immunosuppressants and
N-Acetylcysteine declined since 2009, and were replaced by antifibrotics, under
which patients showed improved survival (p =?0.001). CONCLUSIONS: Our data
provide important insights into baseline characteristics, diagnostic and
management changes as well as outcome data in European IPF patients over time.
TRIAL REGISTRATION: The eurIPFreg and eurIPFbank are listed in
ClinicalTrials.gov( NCT02951416 ).